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. 2020 Oct 30;22(Suppl 1):iv1–iv96. doi: 10.1093/neuonc/noaa200

CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013–2017

Quinn T Ostrom 1,2,#, Nirav Patil 1,3,5,6,#, Gino Cioffi 1,3,5, Kristin Waite 1,3,5, Carol Kruchko 1, Jill S Barnholtz-Sloan 1,3,4,5,6,
PMCID: PMC7596247  PMID: 33123732

Abstract

The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control (CDC) and National Cancer Institute (NCI), is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population. This report contains the most up-to-date population-based data on primary brain tumors (malignant and non-malignant) and supersedes all previous CBTRUS reports in terms of completeness and accuracy. All rates (incidence and mortality) are age-adjusted using the 2000 US standard population and presented per 100,000 population. The average annual age-adjusted incidence rate (AAAIR) of all malignant and non-malignant brain and other CNS tumors was 23.79 (Malignant AAAIR=7.08, non-Malignant AAAIR=16.71). This rate was higher in females compared to males (26.31 versus 21.09), Blacks compared to Whites (23.88 versus 23.83), and non-Hispanics compared to Hispanics (24.23 versus 21.48). The most commonly occurring malignant brain and other CNS tumor was glioblastoma (14.5% of all tumors), and the most common non-malignant tumor was meningioma (38.3% of all tumors). Glioblastoma was more common in males, and meningioma was more common in females. In children and adolescents (age 0-19 years), the incidence rate of all primary brain and other CNS tumors was 6.14. An estimated 83,830 new cases of malignant and non-malignant brain and other CNS tumors are expected to be diagnosed in the US in 2020 (24,970 malignant and 58,860 non-malignant). There were 81,246 deaths attributed to malignant brain and other CNS tumors between 2013 and 2017. This represents an average annual mortality rate of 4.42. The 5-year relative survival rate following diagnosis of a malignant brain and other CNS tumor was 23.5% and for a non-malignant brain and other CNS tumor was 82.4%.

Executive Summary

The Central Brain Tumor Registry of the United States (CBTRUS), in collaboration with the Centers for Disease Control (CDC) and the National Cancer Institute (NCI), is the largest population-based registry focused exclusively on primary brain and other central nervous system (CNS) tumors in the United States (US) and represents the entire US population. The CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017 contains the most up-to-date population-based data on primary brain tumors available through the surveillance system in the US and supersedes all previous CBTRUS reports in terms of completeness and accuracy, thereby providing a current comprehensive source for the descriptive epidemiology of these tumors. All rates are age-adjusted using the 2000 US standard population and presented per 100,000 population.

Incidence

  • The average annual age-adjusted incidence rate of all primary malignant and non-malignant brain and other CNS tumors for the years 2013-2017 was 23.79 per 100,000.

  • This rate was higher in females compared to males (26.31 versus 21.09 per 100,000), slightly higher Blacks compared to Whites (23.88 versus 23.83 per 100,000), and higher in non-Hispanics (of any race) compared to Hispanics (24.23 versus 21.48 per 100,000).

  • The average annual age-adjusted incidence rate of primary malignant brain and other CNS tumors was 7.08 per 100,000.

  • The average annual age-adjusted incidence rate of primary non-malignant brain and other CNS tumors was 16.71 per 100,000.

  • Approximately 29.7% of all primary brain and other CNS tumors were malignant and 70.3% were non-malignant, which makes non-malignant tumors more than twice as common as malignant tumors.

  • The most commonly occurring primary malignant brain and other CNS tumor was glioblastoma (14.5% of all tumors and 48.6% of malignant tumors), and the most common primary non-malignant tumor was meningioma (38.3% of all tumors and 54.5% of non-malignant tumors). Glioblastoma was more common in males, and meningioma was more common in females.

  • In children and adolescents (age 0-19 years), the incidence rate of primary malignant and non-malignant brain and other CNS tumors was 6.14 per 100,000 between 2013 and 2017. Incidence was higher in females compared to males (6.22 versus 6.07 per 100,000), Whites compared to Blacks (6.36 versus 4.83 per 100,000), and non-Hispanics compared to Hispanics (6.42 versus 5.26 per 100,000).

  • An estimated 83,830 new cases of primary malignant and non-malignant brain and other CNS tumors are expected to be diagnosed in the US in 2020. This includes an expected 24,970 primary malignant and 58,860 primary non-malignant tumors.

Mortality

  • There were 81,246 deaths attributed to primary malignant brain and other CNS tumors for the five-year period between 2013 and 2017. This represents an average annual mortality rate of 4.42 per 100,000, and an average of 16,249 deaths per year caused by primary malignant brain and other CNS tumors.

Survival

  • Median observed survival in primary malignant brain and other CNS tumors only was lowest for glioblastoma (8 months) and highest for malignant tumors of the pituitary (139 months, or approximately 11.5 years).

  • The five-year relative survival rate following diagnosis of a primary malignant brain and other CNS tumor was 23.5%. Survival following diagnosis with a primary malignant brain and other CNS tumor was highest in persons age 0-14 years (63.8%), compared to those ages 15-39 years (59.1%) or 40+ years (12.8 %).

  • The five-year relative survival rate following diagnosis of a primary non-malignant brain and other CNS tumor was 82.4%. Survival following diagnosis with a primary non-malignant brain and other CNS tumor was highest in persons age 15-39 years (96.5%), compared to those ages 0-14 years (95.3%) or 40+ years (79.7%).

Introduction

The objective of the CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017 is to provide a comprehensive summary of the current descriptive epidemiology of primary brain and other central nervous system (CNS) tumors in the United States (US) population. The Central Brain Tumor Registry of the United States (CBTRUS) obtained the latest available population-based data on all newly diagnosed primary brain and other CNS tumors from the Centers for Disease Control and Prevention’s (CDC) National Program of Cancer Registries (NPCR), and the National Cancer Institute’s (NCI) Surveillance, Epidemiology, and End Results (SEER) program for diagnosis years 2013-2017. Incidence counts and rates of primary malignant and non-malignant brain and other CNS tumors are presented by histology, sex, age, race, Hispanic ethnicity, and geographic location. Mortality rates calculated using the National Vital Statistics System (NVSS) data from 2013-2017, and both relative survival rates and median survival for selected malignant and non-malignant histologies calculated using SEER and NPCR data for the period 2001-2016, are also presented.

Background

CBTRUS is a unique professional research organization that focuses exclusively on providing high-quality statistical data on the population-based incidence of primary brain and other CNS tumors in the US (for more information on CBTRUS see: http://www.cbtrus.org/about/).1 CBTRUS was incorporated as a nonprofit 501(c)(3) in 1992 following a study conducted by the American Brain Tumor Association (ABTA) to determine the feasibility of a population-based central registry focused on all primary brain and other CNS tumors in the US.

This report represents the twenty-eighth (28 th ) anniversary of CBTRUS and the twenty-third (23 rd ) statistical report published by CBTRUS. For this ninth (9th) report published as a supplement to Neuro-Oncology, the official journal of the Society for Neuro-Oncology (http://www.soc-neuro-onc.org), CBTRUS continues its past efforts to provide the most up-to-date population-based incidence rates for all primary brain and other CNS tumors by behavior (malignant, non-malignant), histology, age, sex, race, and Hispanic ethnicity. These data have been organized by clinically relevant histology groupings and reflect the 2007 World Health Organization (WHO) Classification of Tumours of the Central Nervous System.2,3 These data provide important information for allocation and planning of specialty healthcare services such as clinical trials, disease prevention and control programs, and research activities. These data may also lead to clues that will stimulate research into the causes of this group of diseases, which often result in significant morbidity and mortality.

CBTRUS is currently the only population-based site-specific registry in the US that works in partnership with a public cancer surveillance organization, the CDC’s NPCR, and from which data are directly received through the NPCR Cancer Surveillance System (NPCR-CSS) Submission Specifications mechanism4 under a special agreement. Collection of central (state) cancer data was mandated in 1992 by Public Law 102-515, the Cancer Registries Amendment Act.5 This mandate was expanded to include non-malignant CNS tumors with the 2002 passage of Public Law 107–260, starting January 1, 2004.6 CBTRUS combines the NPCR data with data from the NCI’s SEER program,7 which was established for national cancer surveillance in the early 1970s. All data from NPCR and SEER originate from tumor registrars who adhere to the Uniform Data Standards (UDS) for malignant and non-malignant brain and other CNS tumors as directed by the North American Association of Cancer Registries (NAACCR) (http://www.naaccr.org). Along with the UDS, there are quality control checks and a system for rating each central cancer registry (CCR) to ensure that these data are as accurate and complete as possible. As a surveillance partner, CBTRUS reports high-quality data on brain and other CNS tumors with histological specificity useful to the communities it serves.

The CBTRUS database is comprised of the largest histology-specific aggregation of population-based data limited to the incidence and survival of primary brain and other CNS tumors in the US, and it is likely the largest histology-specific aggregation of primary brain and other CNS tumor cases in the world. Beginning with this report, the CBTRUS database now includes both survival data from 49 CCRs and incidence data from all 51 CCRs in the US. There are several other brain-specific registry systems in existence, including the Canadian Brain Tumor Registry,8 the Austrian Brain Tumor Registry,9 and the Swedish Brain Tumor Registry,10 as well as other population-based epidemiological studies of primary brain and other CNS tumors that cover a smaller population base. Due to the demographics of the US as compared to European countries, CBTRUS includes a greater proportion of cases of primary brain and other CNS tumors in non-White persons. Aggregate information on all cancers from all CCR in the US, including primary brain and other CNS tumors, is available from the United States Cancer Statistics (USCS).11

Technical Notes

Data Collection

CBTRUS does not collect data directly from patients’ medical records. Registration of individual cases (tumors) is conducted by cancer registrars at the institution where diagnosis and/or treatment occur and is then transmitted to the CCR, which further transmits this information to NPCR and/or SEER. Some CCRs also send their data to SEER; data from those CCRs are taken from the NPCR file to eliminate duplicate cases. As noted, data for CBTRUS analyses come from the NPCR and SEER programs. By law, all primary malignant and non-malignant CNS tumors are reportable diseases and CCRs play an essential role in the collection process. Brain and other CNS tumors are reported using the site definition described in Public Law 107-260.6 These data are population-based and represent a comprehensive documentation of all reported cancers diagnosed within a geographic region for the years included in this report.

CBTRUS obtained de-identified incidence data from 52 CCR (48 NPCR and 4 SEER [SEER data available until year 2016 only]) that include cases of malignant and non-malignant (benign and uncertain behaviors) primary brain and other CNS tumors. The population-based CCR include 50 state registries, the District of Columbia, and Puerto Rico (Fig. 1). Data were requested for all newly-diagnosed primary malignant and non-malignant tumors from 2013 to 2017 at any of the following International Classification of Diseases for Oncology, 3rdEdition (ICD-O-3) anatomic sites: brain, meninges, spinal cord, cranial nerves, and other parts of the central nervous system, pituitary and pineal glands, and olfactory tumors of the nasal cavity (Table 1).12

Fig. 1.

Fig. 1

Availability by Central Cancer Registry for SEER and NPCR Incidence (2013-2017, varying) and Survival Data (2001-2016)

Table 1.

Central Brain Tumor Registry of the United States (CBTRUS), Brain and Other Central Nervous System Tumor Site Groupings

Site ICD-O-3a Site Code
Frontal lobe of brain C71.1
Temporal lobe of brain C71.2
Parietal lobe of brain C71.3
Occipital lobe of brain C71.4
Cerebrum C71.0
Ventricle C71.5
Cerebellum C71.6
Brain stem C71.7
Other brain C71.8-C71.9
Overlapping lesion of brain C71.8
Brain, NOS C71.9
Spinal cord and cauda equina C72.0-C72.1
Spinal cord C72.0
Cauda equina C72.1
Cranial nerves C72.2-C72.5
Olfactory nerve C72.2
Optic nerve C72.3
Acoustic nerve C72.4
Cranial nerve, NOS C72.5
Other nervous system C72.8-C72.9
Overlapping lesion of brain and central nervous system C72.8
Nervous system, NOS C72.9
Meninges (cerebral & spinal) C70.0-C70.9
Cerebral meninges C70.0
Spinal meninges C70.1
Meninges, NOS C70.9
Pituitary and craniopharyngeal duct C75.1-C75.2
Pituitary gland C75.1
Craniopharyngeal duct C75.2
Pineal gland C75.3
Olfactory tumors of the nasal cavityb C30.0

a. International Classification of Diseases for Oncology, 3rd Edition, 2000. World Health Organization, Geneva, Switzerland.

b. ICD-O-3 histology codes 9522-9523 only.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NOS, not otherwise specified.

NPCR provided data on 419,321 primary brain and other CNS tumors diagnosed from 2013 to 2017 (Fig. 2). An additional 10,267 primary brain and other CNS tumor case records for the period were obtained from SEER for primary brain and other CNS tumor case records from 2013 to 2016 for Connecticut, Hawaii, Iowa, and New Mexico only. These data were combined into a single dataset of 429,588 records for quality control. A total of 11,821 records (2.71%) were deleted from the final analytic dataset for one or more of the following reasons:

Fig. 2.

Fig. 2

Overview of CBTRUS Data Cleaning Workflow, NPCR 2013-2017 and SEER 2013-2016

  • Records with ICD-O-3 behavior code of /2 (Indicates in situ cases, which is not a relevant classification for brain and other CNS tumors).

  • Records with an invalid site/histology combination according to the CBTRUS histology grouping scheme

  • Possible duplicate records that included a less accurate reporting source than microscopic confirmation, also referred to as histologic confirmation (e.g. radiographic versus microscopic confirmation), possible duplicate record for recurrent disease, or errors in time sequence of diagnosis

  • Possible duplicate records for bilateral vestibular schwannoma or meningioma that were merged to one paired-site record.

The final analytic dataset had 417,767 records, which included 415,411 records from the 50 state CCR and the District of Columbia used in the analytic dataset, and an additional 2,356 records from Puerto Rico. Records from Puerto Rico are included only in a supplementary analysis (See Supplemental Material), and these cases are not included in the overall statistics presented in this report.

Age-adjusted incidence rates per 100,000 population for the entire US for selected other cancers were obtained from the United States Cancer Statistics (USCS), produced by the CDC and the NCI, for the purpose of comparison with brain and other CNS tumor incidence rates.11 This database includes both NPCR and SEER data and represents the entire US population.

De-identified survival data for malignant brain and other CNS tumors were obtained from the US Cancer Statistics program for 45 NPCR registries for the years 2001 to 2016 and for non-malignant brain and other CNS tumors for the years 2004 to 2016. This dataset provides population-based information for 93.6% of the US population and is a subset of the data used for the incidence calculations presented in this report. Survival information is derived from both active and passive follow-up.

Mortality data used in this report are from the National Center for Health Statistics’ (NCHS) National Vital Statistics System (NVSS) and include deaths where primary brain or other CNS tumor was listed as primary cause of death on the death certificate for individuals from all 50 states and the District of Columbia. These data were obtained from NVSS13 (includes death certification data for 100% of the US population) for malignant brain and other CNS tumors and comparison via SEER*Stat (for malignant brain tumors and comparison cancers). NVSS data are not collected through the cancer registration system. These data represent the primary cause of death listed on each individual death certificate, and as a result, deaths in persons with cancer may be recorded as non-cancer deaths.

Definitions

Measures in Surveillance Epidemiology

The CBTRUS Report presents the following population-based measures: incidence rates, mortality rates, observed survival (median survival time and hazard ratios) and relative survival rates (for more information on definitions of terms and measures used see: https://cbtrus.org/cbtrus-glossary/).

Variable Completeness in Cancer Registration

Obtaining the most accurate and complete cancer registration data possible is essential to generate accurate population-level statistics to guide public health planning. Agencies such as NAACCR and IACR have developed stringent standards for evaluation of cancer registry data quality, and evaluate each specific registry by multiple metrics before including it in analytic datasets.14,15 While many measures of quality and completeness are assessed across all cancer sites, some variables are pertinent only to specific sites and/or histologies and require special care. In the case of primary brain and other CNS tumors, variables such as WHO grade are not relevant to histologies (e.g. many tumors of the pituitary) that are not assigned a WHO grade. Variables like WHO grade may also not be expected to be found in the patient record for those who had their diagnosis confirmed via radiography as compared to histological examination. The report evaluates the completeness of multiple variables, including: WHO grade, radiation treatment, and chemotherapeutic treatment.

Classification by Histology

There are over 100 histologically distinct types of primary CNS tumors, each with its own spectrum of clinical presentations, treatments, and outcomes. These histologies are reviewed periodically by neuropathologists and published by the World Health Organization (WHO) in Classification Reports known as “Blue Books”. Blue Books are published for all cancer sites by WHO and utilize the International Classification of Diseases for Oncology, third edition (ICD-O-3) for assignment of histology, behavior, and site codes. This report uses the 2007 WHO Classification of Tumors of the Central Nervous System to guide its reporting, the most recent being the 2012 CBTRUS Histology Grouping (Table 2). The ICD-O-3 codes in this current CBTRUS Grouping 12 may include morphology codes that were not previously reported to CBTRUS.16 In this report, incidence rates are provided for major histology groupings and for specific histologies found in the 2012 CBTRUS Histology Grouping. CBTRUS will be using a Histology Grouping according to 2016 WHO Classification of CNS Tumours in its 2021 Report at which time the CBTRUS Histology Grouping will be updated.

Table 2.

Central Brain Tumor Registry of the United States (CBTRUS), Brain and Other Central Nervous System Tumor Histology Groupings, Overall and by Behavior

Histology ICD-O-3a Histology Code ICD-O-3a Histology and Behavior Codeb
Malignant Non-Malignant
Tumors of Neuroepithelial Tissue
Pilocytic astrocytoma* 9421, 9425c 9421/1, 9425/3d None
Diffuse astrocytoma* 9400, 9410, 9411, 9420 9400/3, 9410/3, 9411/3, 9420/3 None
Anaplastic astrocytoma* 9401 9401/3 None
Unique astrocytoma variants* 9381, 9384, 9424 9381/3, 9424/3 9384/1
Glioblastoma* 9440, 9441, 9442/3d 9440/3, 9441/3, 9442/3 None
Oligodendroglioma* 9450 9450/3 None
Anaplastic oligodendroglioma* 9451, 9460 9451/3, 9460/3 None
Oligoastrocytic tumors* 9382 9382/3 None
Ependymal tumors* 9383, 9391, 9392, 9393, 9394 9391/3, 9392/3, 9393/3 9383/1, 9394/1
Glioma malignant, NOS* 9380, 9431c, 9432c 9380/3, 9431/1, 9432/1 None
Choroid plexus tumors 9390 9390/3 9390/0,1
Other neuroepithelial tumors* 9363, 9423, 9430, 9444 9423/3, 9430/3 9363/0, 9444/1
Neuronal and mixed neuronal-glial tumors* 8680, 8681, 8690, 8693, 9412, 9413, 9442/1e, 9492 (excluding site C75.1), 9493, 9505, 9506, 9509, 9522, 9523 8680/3, 8693/3, 9505/3, 9509/3, 9522/3, 9523/3 8680/0,1, 8681/1, 8690/1, 8693/1, 9412/1, 9413/0, 9442/1, 9492/0 (excluding site C75.1), 9493/0, 9505/1, 9506/1, 9509/1,
Tumors of the pineal region 9360, 9361, 9362, 9395c 9362/3, 9395/3c 9360/1, 9361/1
Embryonal tumors 8963, 9364, 9470-9474, 9480, 9490, 9500-9502, 9508 8963/3, 9364/3, 9470/3, 9471/3, 9472/3, 9473/3, 9474/3, 9480/3, 9490/3, 9500/3, 9501/3, 9502/3, 9508/3 9490/0
Medulloblastoma 9470, 9471, 9472, 9474 9470/3, 9471/3, 9472/3, 9474/3. None
Primitive neuroectodermal tumors 9473 9473/3. None
Atypical teratoid rhabdoid tumors 9508 9508/3. None
Other embryonal histologies 8963, 9364, 9480, 9490, 9500, 9501, 9502 8963/3, 9364/3, 9480/3, 9490/3, 9500/3, 9501/3, 9502/3 9490/0
Tumors of Cranial and Spinal Nerves
Nerve sheath tumors 9540, 9541, 9550, 9560, 9561, 9570, 9571 9540/3, 9560/3, 9561/3, 9571/3 9540/0,1, 9541/0, 9550/0, 9560/0,1, 9570/0, 9571/0
Vestibular schwannoma 9560 (C72.4 and C72.5 only) None 9560/0 (C72.4 and C72.5 only)
Other tumors of cranial and spinal nerves 9562 None 9562/0
Tumors of Meninges
Meningioma 9530-9534, 9537-9539 9530/3, 9538/3, 9539/3 9530/0,1, 9531/0, 9532/0, 9533/0, 9534/0, 9537/0, 9538/1, 9539/1
Mesenchymal tumors 8324, 8800-8806, 8810, 8815, 8824, 8830, 8831, 8835, 8836, 8850-8854, 8857, 8861, 8870, 8880, 8890, 8897, 8900-8902, 8910, 8912, 8920, 8921, 8935, 8990, 9040, 9136, 9150, 9170, 9180, 9210, 9241, 9260, 9373 8800/3, 8801/3, 8802/3, 8803/3, 8804/3, 8805/3, 8806/3, 8810/3, 8815/3, 8830/3, 8850/3, 8851/3, 8852/3, 8853/3, 8854/3, 8857/3, 8890/3, 8900/3, 8901/3, 8902/3, 8910/3, 8912/3, 8920/3, 8921/3, 8990/3, 9040/3, 9150/3, 9170/3, 9180/3, 9260/3 8324/0, 8800/0, 8810/0, 8815/0, 8824/0,1, 8830/0,1, 8831/0, 8835/1, 8836/1, 8850/0,1, 8851/0, 8852/0, 8854/0, 8857/0, 8861/0, 8870/0, 8880/0, 8890/0,1, 8897/1, 8900/0, 8920/1, 8935/0,1, 8990/0,1, 9040/0, 9136/1, 9150/0,1, 9170/0, 9180/0, 9210/0, 9241/0, 9373/0
Primary melanocytic lesions 8720, 8728, 8770, 8771 8720/3, 8728/3, 8770/3, 8771/3 8728/0,1, 8770/0, 8771/0
Other neoplasms related to the meninges 9161, 9220, 9231, 9240, 9243, 9370-9372, 9535 9220/3, 9231/3, 9240/3, 9243/3, 9370/3, 9371/3, 9372/3 9161/1, 9220/0,1, 9535/0
Lymphomas and Hematopoietic Neoplasms
Lymphoma 9590, 9591, 9596, 9650-9655, 9659, 9661-9665, 9667, 9670, 9671, 9673, 9675, 9680, 9684, 9687, 9690, 9691, 9695, 9698, 9699, 9701, 9702, 9705, 9714, 9719, 9728, 9729 9590/3, 9591/3, 9596/3, 9650/3, 9651/3, 9652/3, 9653/3, 9654/3, 9655/3, 9659/3, 9661/3, 9662/3, 9663/3, 9664/3, 9665/3, 9667/3, 9670/3, 9671/3, 9673/3, 9675/3, 9680/3, 9684/3, 9687/3, 9690/3, 9691/3, 9695/3, 9698/3, 9699/3, 9701/3, 9702/3, 9705/3, 9714/3, 9719/3, 9728/3, 9729/3 None
Other hematopoietic neoplasms 9727, 9731, 9733, 9734, 9740, 9741, 9750-9758, 9760, 9766, 9823, 9826, 9827, 9832, 9837, 9860, 9861, 9866, 9930, 9970 9727/3, 9731/3, 9733/3, 9734/3, 9740/3, 9741/3, 9750/3, 9754/3, 9755/3, 9756/3, 9757/3, 9758/3, 9760/3, 9823/3, 9826/3, 9827/3, 9832/3, 9837/3, 9860/3, 9861/3, 9866/3, 9930/3 9740/1, 9751/1, 9752/1, 9753/1, 9766/1, 9970/1
Germ Cell Tumors and Cysts
Germ cell tumors, cysts and heterotopias 8020, 8440, 9060, 9061, 9064, 9065, 9070-9072, 9080-9085, 9100, 9101 8020/3, 8440/3, 9060/3, 9061/3, 9064/3, 9065/3, 9070/3, 9071/3, 9072/3, 9080/3, 9081/3, 9082/3, 9083/3, 9084/3, 9085/3, 9100/3, 9101/3 8440/0, 9080/0,1, 9084/0
Tumors of Sellar Region
Tumors of the pituitary 8040, 8140, 8146, 8246, 8260, 8270-8272, 8280, 8281, 8290, 8300, 8310, 8323, 9492 (Site C75.1 only), 9582 8140/3, 8246/3, 8260/3, 8270/3, 8272/3, 8280/3, 8281/3, 8290/3, 8300/3, 8310/3, 8323/3 8040/0,1, 8140/0,1, 8146/0, 8260/0, 8270/0, 8271/0, 8272/0, 8280/0, 8281/0, 8290/0, 8300/0, 8310/0, 8323/0, 9492/0 (site C75.1 only), 9582/0
Craniopharyngioma 9350, 9351, 9352 None 9350/1, 9351/1, 9352/1
Unclassified Tumors
Hemangioma 9120-9123, 9125, 9130, 9131, 9133, 9140 9120/3, 9130/3, 9133/3, 9140/3 9120/0, 9121/0, 9122/0, 9123/0, 9125/0, 9130/0,1, 9131/0, 9133/1
Neoplasm, unspecified 8000-8005, 8010, 8021 8000/3, 8001/3, 8002/3, 8003/3, 8004/3, 8005/3, 8010/3, 8021/3 8000/0,1, 8001/0,1, 8005/0, 8010/0
All other 8320, 8452, 8710, 8711, 8713, 8811, 8840, 8896, 8980, 9173, 9503, 9580 8320/3, 8710/3, 8711/3, 8811/3, 8840/3, 8896/3, 8980/3, 9503/3, 9580/3 8452/1, 8711/0, 8713/0, 8811/0, 8840/0, 9173/0, 9580/0

a. International Classification of Diseases for Oncology, 3rd Edition, 2000. World Health Organization, Geneva, Switzerland.

b. See the CBTRUS website for additional information about the specific histology codes included in each group: http://www.cbtrus.org.

c. Histology included starting with diagnosis year 2015.

d. Morphology 9442/3 only.

e. Morphology 9442/1 only.

* All or some of this histology is included in the CBTRUS definition of gliomas, including ICD-O-3 histology codes 9380-9384, 9391-9460.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NOS, not otherwise specified.

Gliomas are tumors that arise from glial or precursor cells and include astrocytoma (including glioblastoma), oligodendroglioma, ependymoma, oligoastrocytoma (mixed glioma), and a few rare histologies. Because there is no standard definition for glioma, CBTRUS defines glioma as ICD-O-3 histology codes 9380-9384, and 9391-9460 as starred in Table 2. It is also important to note that the statistics for lymphomas and hematopoietic neoplasms contained in this report refer only to those lymphomas and hematopoietic neoplasms that arise in the brain and other CNS ICD-O-3 topography codes.

This report also utilizes the International Classification of Childhood Cancer (ICCC) grouping system for pediatric brain and other CNS tumors. ICCC categories for this report were generated using the SEER Site/Histology ICCC-3 Recode17 based on the ICCC, Third edition18 and 2007 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues19 (See Supplementary Table 1 for more information on this classification scheme). The ICCC was developed in order to provide a standard classification of childhood tumors for comparing incidence and survival across regions and time periods. As shown, the Supplementary Table 8 age-group category total, age 0-19 year age-group count, and age-specific and age-adjusted rates are equivalent to those presented throughout this report, even though the histology grouping scheme differs from that used by CBTRUS.

Classification by Behavior

Primary brain and other CNS tumors can be broadly classified in non-malignant (ICD-O-3 behavior codes of /0 for benign and /1 for uncertain) and malignant (ICD-O-3 behavior code of /3) (Table 2). Collection of central (state) cancer data was mandated in 1992 by Public Law 102-515 for all primary malignant tumors (ICD-O-3 behavior code of /3) (Table 2), the Cancer Registries Amendment Act.5 This mandate was expanded to include non-malignant brain and other CNS tumors (ICD-O-3 behavior code of /0 and /1) with the 2002 passage of Public Law 107–260, starting January 1, 2004.6 Collection of metastatic tumors are not included in these public laws. CBTRUS reports data on all brain and other CNS tumors irrespective of behavior, whereas many reporting organizations may only publish rates for primary malignant brain and other CNS tumors due to the original mandate that focused only on primary malignant tumors, sometimes using the term cancer to broadly identify these tumors in their reports. These differences in definition therefore influence the direct comparison of published rates.

Classification by WHO Grade

Unlike other types of cancer which are staged according to the American Joint Commission of Cancer (AJCC) Collaborative Staging (CS) schema, primary brain and other CNS tumors are not staged. They are classified according to the WHO 2000 Classification of Tumours of the Central Nervous System20 which assigns a grade (grade I through grade IV) based on predicted clinical behavior. Though the WHO classification scheme was also updated in 20072 and 201621 these updated schema will not be fully implemented by US CCR until collection year 2018 or reporting year 2021. Updates made in 2007 may affect diagnostic practices used in characterization of individual tumors included in this report, though the newest revision would not affect any cases included in this report. With the increased recognition of the value of biomarkers for specific brain tumor histologies in classification, the WHO Classification of Tumours of the Central Nervous System has included biomarkers in its 2016 revision. However, implementing the collection of these markers in cancer registration is multi-faceted and includes an ongoing educational and training component. Collection of these markers began in the US on January 1, 2018.

The WHO grading assignments are recorded by cancer registrars as Collaborative Stage Site-Specific Factor 1 - WHO Grade Classification as directed in the AJCC Chapter 72 on Brain and Spinal Cord 22 This variable has been a required component of cancer registry data collection for brain and other CNS tumors since 2004 for SEER registries, and since 2011 for NPCR registries, and completeness of this variable has improved significantly over time.23,24 Completeness of this variable is defined as having a value equal to WHO grade I, II, III, or IV. Cases where WHO grade is marked as not applicable or not documented are considered incomplete. It is not possible to conclusively determine WHO grade, which is based on the appearance of tumor cells, when a tumor is radiographically confirmed only. Some tumor types (including tumors of the pituitary and lymphomas) are often not assigned a WHO grade. This information may also be assigned but not included in the pathology report.

Anatomic Location of Tumor Sites

Various terms are used to describe the regions of the brain and other CNS. The specific sites used in this report are based on the topography codes found in ICD-O-3 and are broadly based on the categories and site codes defined in the SEER Site/Histology Validation List.25 See Table 1 for an overview of CBTRUS primary site groupings.

Statistical Methods

Statistical Software

Counts, means, medians, rates, ratios, proportions, and other relevant statistics were calculated using R 4.0 statistical software26 and/or SEER*Stat 8.3.6.27 Figures and tables were created in R 4.0.0 using the following packages: knitr, flextable, officer, orca, plotly, SEER2R, sf, survminer, tigris, and tidyverse.28-37 Rates are suppressed when counts are fewer than 16 within a cell but included in totals, except when data are suppressed from only one cell to prevent identification of the number in the suppressed cell. NOTE: reported percentages may not add up to 100% due to rounding.

Variable Definitions

CBTRUS presents statistics on the pediatric and adolescent age- group 0-19 years as suggested by clinicians, for clinical relevance. However, the 0-14 years age-group is a standard age category for childhood cancer used by other cancer surveillance organizations and has been included in this report for consistency and comparison purposes.

Race categories in this report are all races, White, Black, American Indian/Alaskan Native (AIAN), and Asian/Pacific Islander (API). Other race, unspecified, and unknown race are included in statistics that are not race-specific. Hispanic ethnicity was defined using the NAACCR Hispanic Identification Algorithm, version 2, data element, which utilizes a combination of cancer registry data fields (Spanish/Hispanic Origin data element, birthplace, race, and surnames) to directly and indirectly classify cases as Hispanic or non-Hispanic.38 The United States Department of Agriculture’s 2013 Rural Urban Continuum Codes (RUCCs), which classify counties by population size and proximity to a metropolitan area, were used to classify counties either as rural or urban (rural RUCC 4-9; urban RUCC 1-3).39

Estimation of Incidence Rates and Incidence Rate Ratios

Population data for each geographic region were obtained from the SEER program website40 for the purpose of rate calculation. All rates presented in this statistical report are age-adjusted. Crude incidence rates are calculated by dividing the total number of cases by the total population and cannot be compared to crude rates from other populations where the age distribution is different. Age-adjustment is a technique that is used to enable comparison between groups with different age distributions, such as rates between different states. Rates that have been age-adjusted are estimates of what the crude rate would be if the age distribution was equivalent to a standard population. Average annual age-adjusted incidence rates (AAAIR), average annual age-adjusted mortality rates and 95% confidence intervals (95% CI) were estimated per 100,000 population, based on one-year age groupings and standardized to the 2000 US standard population.41 The age distribution of the 2000 US standard population is presented in Supplementary Table 2. Combined populations for the regions included in this report are also presented in Supplementary Table 3, Supplementary Table 4, and Supplementary Table 5.

Incidence rate ratios (IRR) were generated based on these age-adjusted incidence rates. These IRR were used to compare groups, using the formulas described by Fay et al. to calculate p-values.42 Incidence rate ratios were considered statistically significantly different when the p-value was less than 0.05.

When comparing two rates to one another, it is important to consider whether they are truly different or whether the difference in the estimates may be due to random error. Two methods are used in this report for determining whether two values are ‘significantly different,’ meaning whether the evidence meets a level of strength (usually a 5% chance of error) where the difference can be assumed to not be due to random error. The first is through the use of a 95% confidence interval (CI), which were calculated for all presented rates. A 95% CI is a range around an estimate, which, if sampling of the population were to be repeated, should contain the ‘true’ value for the population 95% of the time. If the CI of two estimates do not overlap, these values are considered significantly different with a less than 5% probability of happening by chance. The second method used is the calculation of p-values. A p-value is the probability of finding the observed or more extreme results by chance alone, and a p-value of <0.05 (or <5% chance of results being due to chance) is conventionally used as a cut-off for considering a value statistically significant. Therefore, a p-value <0.0001 could be interpreted as meaning the observed value (or a more extreme value) had a <0.01% chance of occurring by chance alone, and the difference can be considered statistically significant at the 0.01% level.

Estimation of Expected Numbers of Brain and Other CNS Tumors in 2020 and 2021

Estimated numbers of expected primary malignant and non-malignant brain and other CNS tumors were calculated for 2020 and 2021. To project estimates of newly diagnosed brain and other CNS tumors in 2020 and 2021, age-adjusted annual brain tumor incidence rates were generated for 2000-2017 for malignant tumors, and 2006-2017 for non-malignant tumors. These were generated by state, age, and histologic type. Joinpoint 4.7.0.043 was used to fit regression models to these incidence rates,44 which were used to predict numbers of cases in future years using the parameter from the selected models. Joinpoint regression allows for multiple lines to be fitted to incidence data across time, rather than assuming a consistent trend across the whole period. The points where these lines intersect are called ‘joinpoints’. The models allowed for a maximum of two joinpoints (one for non-malignant tumors), a minimum of three observations from a joinpoint to either end of the data, and a minimum of three observations between joinpoints.45 Modified Bayesian Information Criterion procedures included in Joinpoint were used to select the best fitting model. The overall totals presented are based on total malignant and non-malignant incidence, and the presented stratified rates may not add up to these totals. Estimated numbers of cases are highly dependent on input data. Different patterns of incidence within strata can significantly affect the projected estimates, especially when the number of cases within a stratum is low. For state-specific projections, a model with no joinpoints was used to generate predictions as annual variability within some states was extremely high. As a result, strata-specific estimates may not equal the total estimate presented. Caution should be used when utilizing these estimates.

Estimation of Mortality Rates for Brain and Other CNS Tumors

Age-adjusted mortality rates for deaths resulting from all primary malignant brain and other CNS tumors were calculated using the mortality data available in SEER*Stat Online Database provided by NCHS from death certificates per 100,000 population.13 These data were available for 50 states and the District of Columbia only. In addition to the total age-adjusted rate for the US, age-adjusted rates are presented by sex and state.

Estimation of Incidence-Based Mortality Rates for Brain and Other CNS Tumors

US cancer registry vital status are usually derived from death certificate data, which are coded using the ICD classification scheme. While this scheme for estimating mortality rates classifies deaths due to a brain tumor by site of tumor, it does not allow for partitioning by specific histology. Incidence-based mortality is a method that estimates mortality using population-level cancer registry data, rather than death certificates, and as a result allows for partitioning by additional variables abstracted as part of the process of cancer registration.46 Incidence-based age-adjusted mortality rates for deaths resulting from all primary malignant brain and other CNS tumors were calculated using the data from 18 central cancer registries included in the SEER 18 47 available in SEER*Stat Online from diagnosis years 2008-2017. These registries represent 28% of the US population and are a subset of those registries included in the overall CBTRUS analytic dataset. Caution must be used in interpreting these results, as they can be affected by factors, such as reporting delay and lead-time bias, which generally do not affect mortality rates estimated from death certification data.

Survival Measures Used In This Report

Relative Survival Rates

Relative survival is a way of presenting survival patterns at a population level that is commonly used in cancer statistics reporting. This measure is presented as a percent of people living a period of time (e.g. five years after their diagnosis). Relative survival is calculated using observed survival (the percentage of people diagnosed with cancer that live to the period of time for which relative survival is calculated) and estimated survival (the percent of the general population of the same age that is expected to survival after being followed for that same period of time). This adjustment for estimated survival attempts to exclude deaths that would otherwise have occurred due to other causes. For example, if five-year relative survival for glioblastoma is 5%, that means that out of every hundred people diagnosed with glioblastoma five will be living five years after diagnosis, excluding deaths due to other causes.

SEER*Stat 8.3.6 statistical software was used to estimate one-, two-, three-, four-, five-, and ten-year relative survival rates for primary malignant and non-malignant brain and other CNS tumor cases diagnosed between 2004-2016 in 45 NPCR CCRs. This software utilizes life-table (actuarial) methods to compute survival estimates and accounts for current follow-up. Second or later primary tumors, cases diagnosed at autopsy, cases in which race or sex is coded as other or unknown, and cases known to be alive but for whom follow-up time could not be calculated, were excluded from survival data analyses.

Observed Survival with Median Survival Times and Adjusted Hazard Ratios

Median survival time is another way of presenting survival patterns in a population. This measure is calculated using a method called a Kaplan Meier estimator, which is used to estimate the proportion of individuals within a set that are alive at particular time points. The median observed survival time is the point at which exactly 50% of individuals have either died or been ‘censored’, meaning that their further survival status is unknown beyond a particular date.

Median observed survival time for all primary malignant brain and other CNS tumors diagnosed between 2001-2016 in 45 NPCR CCRs was calculated by histology using the Kaplan Meier method in R 4.0.0 statistical software26 overall, as well as by three major age groups (0-14 years old, 15-39 years old, and 40+ years old). Second or later primary tumors, cases diagnosed at autopsy, cases in which race or sex is coded as other or unknown, and cases known to be alive but for whom follow-up time could not be calculated, were excluded from survival data analyses.

The hazard ratio is a measure of how often an event (in this case, death) occurs in one group as compared to another group over time. A hazard ratio of one means that survival is equal in both groups, while a ratio of less than one means that observed survival is better in the comparison group than in the reference group. A ratio of greater than one means that survival is worse in the comparison group than in the reference group.

Cox proportional hazard models were used to test associations between demographic factors and overall observed survival by histology for malignant brain and other CNS tumors. All models were adjusted for age at diagnosis group (0-14 years [reference], 15-39 years, 40+ years), sex (male [reference], female), race (White [reference], Black, AIAN, API), and ethnicity (non-Hispanic [reference], Hispanic). These models were used to estimate hazard ratios associated with each group and corresponding 95% confidence intervals and p-values. Adjusted estimates included all covariates (age at diagnosis, sex, race, and ethnicity) a priori, regardless of individual significance level. The proportional hazards assumption was tested separately by histology, and residuals were examined for all variables.

Estimation of Incidence Time Trends

Joinpoint 4.7.0.043 was used to estimate incidence time trends and generate annual percentage changes (APC) and 95% CI. Rather than calculating a single consistent slope of change over an entire time period, joinpoint allows for points where the slope of the trend can change during the time period (joinpoints). This method starts with a model that assumes one consistent trend over time, and tests whether the addition of these ‘joinpoints’ results in a model which has a fit that represents a statistically significant improvement over the model with no joinpoints. These models are tested through use of Monte Carlo permutations, e.g. the program repeats the same analysis multiple times using random samples to identify the ‘true’ proportion of times that a comparison is statistically significant. The models allowed for a maximum of three joinpoints (two for non-malignant tumors), a minimum of three observations from a joinpoint to either end of the data, and a minimum of three observations between joinpoints.45

APC is the average percent change in incidence per year over the period included in the trend segment. Time trends analysis methods were used to estimate if the APC was significantly different from 0% (meaning no change in incidence from year to year). The 95% CI is a range around an estimate that, if sampling of the population were to be repeated, should contain the ‘true’ value for the population 95% of the time. If the 95% CI contains zero, one cannot be confident that the ‘true’ population APC value is significantly different from 0%. The joinpoint regression program fits a linear regression to annual incidence rates to test significance of changes overtime, with different trends lines connected at ‘joinpoints’ where there are changes in the direction of incidence trends. The best fitting model was determined through permutation tests, with a minimum of three observations required between two joinpoints, as well as a minimum of three observations required between a joinpoint and either end of the data.

Brain Tumor Definition Differences

Currently, NPCR, SEER, and NAACCR report primary brain and other CNS tumors differently from CBTRUS. The definition of primary brain and other CNS tumors used by these organizations in their published incidence and mortality statistics includes tumors located in the following sites with their ICD-O-3 site codes in parentheses: brain, meninges, and other central nervous system tumors (C70.0-9, C71.0-9, and C72.0-9), but excludes lymphoma and leukemia histologies (9590-9989) from all brain and other CNS sites.3 In contrast, CBTRUS reports data on all tumor morphologies located within the Consensus Conference site definition including lymphoma and other hematopoietic histologies, tumors of the pituitary, as well as olfactory tumors of the nasal cavity [C30.0 (9522-9523)].16 Additionally, CBTRUS reports data on all primary brain and other CNS tumors irrespective of behavior, whereas many reporting organizations may only publish rates for malignant brain and other CNS tumors due to the original mandate that focused only on malignant tumors, sometimes using the term cancer to broadly identify these tumors in their reports. These differences in definition therefore influence the direct comparison of published rates.

CBTRUS is currently engaged in ongoing collaboration with other cancer registry reporting groups, including SEER, to harmonize brain tumor reporting definitions. Therefore, it is likely that these reporting differences will cease to exist in the future.

Pilocytic astrocytoma is clinically considered and classified as a Grade I, non-malignant (ICD-O-3 behavior code of /1) tumor by the World Health Organization (WHO) guidelines for brain and other central nervous system (CNS) tumors.48 For the purposes of cancer registration, these tumors have historically been reported as malignant (ICD-O-3 behavior code of /3) tumors both in the US and by the International Agency for Research on Cancer and International Association of Cancer Registries. 49,50 Classification of these tumors as malignant has been followed by CBTRUS in its reporting unless otherwise stated. This practice does not correlate with clinical classification and presents a challenge to correctly report population-based incidence and survival patterns associated with these tumors. Please see a recent publication for additional discussion of the effect of this classification on cancer incidence and survival reporting.51

In the US, cancer registries and surveillance groups only collect data on primary CNS tumors (meaning tumors that originate within the brain and spinal cord) and do not collect data on tumors that metastasize to the brain or spinal cord from other primary sites. As a result, only primary brain and other CNS tumors are included in this report.

Data Interpretation

CBTRUS works diligently to support the broader surveillance efforts aimed at improving the collection and reporting of primary brain and other CNS tumors. CCR data provided to NPCR and SEER and, subsequently, to CBTRUS vary from year-to-year due to ongoing updates in collection and data refinement aimed to improve completeness and accuracy. Therefore, it is important to note that data from previous CBTRUS Reports cannot be compared to data in this current report, CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017. This current report supersedes all previous reports in terms of coverage of the US population with the most up-to-date population-based information available, making these data the most accurate and timely to reference.

Several factors should be considered when interpreting the data presented in this report:

  • Incident counts of cases represent individual tumors and not persons. A single person could contribute multiple counted primary tumor cases to the data included in this report. The 417,767 tumors cases, from 50 state CCR and the District of Columbia, included in this report came from 409,965 individuals. Of these 409,965 individuals, there were 5,174 individuals (1.3%) that contributed information on multiple tumors (two or more) to this report.

  • Random fluctuations in average annual rates are common, especially for rates based on small case counts. The CBTRUS policy to suppress data presentation for cells with counts of fewer than 16 cases is consistent with the NPCR policy.

  • A 2007 policy change guiding the Veterans Health Administration (VHA) resulted in probable underreporting of cancer data—especially for men—to CCRs. Recent investigations suggest that underreporting for VHA facilities has diminished over time, and that the Veterans Affairs Central Cancer Registry (VACCR) now captures approximately 87-90% of cases.52,53 It is important to note that improved reporting to VACCR does not necessarily mean that reporting to the state CCR has improved, and the VACCR does not submit data to NPCR or SEER.

  • Delays in reporting and late ascertainment are a reality and a known issue influencing registry completeness and, consequently, rate underestimations occur, especially for the most recent years.54,55 The SEER and NPCR programs allow for reporting delay of up to 22-23 months prior to public data release, but additional cases may still be discovered after that point.56 On average across all cancer sites, the submissions for the most recent diagnosis year are approximately 4% lower than the total number of cases that will eventually be submitted. This problem may be even more likely to occur in the reporting of non-malignant brain and other CNS tumors, where reporting often comes from non-hospital-based sources, such as free standing clinics or outpatient facilities.

  • Type of diagnostic confirmation may also lead to increased reporting delay, with histologically confirmed tumors being subject to less reporting delay than radiographically confirmed tumors. In 2016, a study assessing the incidence of non-malignant brain and other CNS tumors corroborated the large variation in incidence between CCRs reported in this statistical report.57 The reasons for this variation remain inconclusive but what is consistently noted is the correlation between high incidence and high proportion of non-malignant cases collected without microscopic confirmation or surgery, in other words, clinically diagnosed cases of non-malignant brain tumors. At this current time, given the variation across CCRs, there is potential evidence of underreporting of non-malignant brain and other CNS tumors, the extent to which cannot be quantified.57

  • Population estimates used for denominators affect incidence rates. CBTRUS has utilized population estimates based on the 2000 US Census for calculation of incidence and mortality rates in this report, as is standard practice in US cancer registry reporting.58,59

CBTRUS editing practices are reviewed, revised, and conducted yearly. These practices are aimed at refining the data for accuracy and clinical relevance and play a role in interpreting these report data. Exclusion of site and histology combinations considered invalid by the consulting neuropathologists who revised the CBTRUS site/histology validation list in 2012 may have the impact of underestimating the incidence of brain and other CNS tumors. Editing changes, such as the Multiple Primary and Histology Rules issued in 2007 and revised in 2018,60,61 also incorporate updates to the cancer registration coding rules that influence case ascertainment and data collection.3

Supplemental Data

CBTRUS has made supplemental additional figures and tables available. These materials are noted in the text as Supplementary Tables and Figures.

Results

Incidence and Mortality in Comparison to Other Common Cancer Types in the US

Average annual age-adjusted incidence rates for primary brain and other CNS tumors for the period from 2013-2017 and a selection of common cancers (USCS, also from 2013-2017) in the US are presented by age in Fig. 3. Incidence rates stratified by sex are presented by age in Supplementary Figure 1. Please see Supplementary Table 6 for incidence rates of comparison cancers.

Fig. 3.

Fig. 3

Average Annual Age-Adjusted Incidence Ratesa with 95% Confidence Intervals of All Primary Brain and Other CNS Tumors in Comparison To Top Eight Highest Incidence Cancers for Children Age 0-14 Years, Adolescents and Young Adults Age 15-39 Years, and Older Adults Age 40+ Years, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER 2013-2017

  • Brain and other CNS tumors (both malignant and non-malignant) were the most common cancer site in persons age 0-14 years, with an AAAIR of 5.83 per 100,000 population. Brain and other CNS tumors were the most common cancer in both males and females in this age group.

  • Leukemia was the 2nd most common neoplasm in persons age 0-14 years, with an AAAIR of 4.99 per 100,000 population. Leukemia was the 2nd most common cancer in both males and females in this age group.

  • Testicular cancer was the most common cancer in males age 15-39 years, with an AAAIR of 10.87 per 100,000.

  • Breast cancer was the most common cancer among females age 15-39 years, with an AAAIR of 19.59 per 100,000.

  • Brain and other CNS tumors (both malignant and non-malignant) among those age 15-39 years had an AAAIR of 11.54 per 100,000 population. These tumors were the 3rd most common cancer overall, the 2nd most common cancer in males in this age group, and the 3rd most common cancer in females in this age group.

  • Breast cancer was the most common cancer among females age 40+ years in the US, with AAAIR of 271.91 per 100,000 population.

  • The most common cancer among males was prostate cancer, which had an incidence rate of 240.13 per 100,000.

  • Brain and other CNS tumors (both malignant and non-malignant) were the 8th most common cancer among persons age 40+ years with an AAAIR of 42.85 per 100,000 population. These tumors were the 9th most common cancer among males and the 5th most common cancer among females in this age group.

Average annual age adjusted mortality rate (AAAMR) for primary malignant brain and other CNS tumors (2013-2017), a selection of common cancers, and the top three non-cancer causes of death in the US are presented by age in Fig. 4. Mortality rates for males only and females only are presented by age in Supplementary Figure 2. Please see Supplementary Table 7 for mortality rates due to comparison cancers and other non-cancer conditions.

Fig. 4.

Fig. 4

Average Annual Age-Adjusted Mortality Ratesa with 95% Confidence Intervals of All Primary Brain and Other CNS Tumors in Comparison To Top Five Causes of Cancer Death and Top Three Non-Cancer Causes of Death (COD) for Children Age 0-14 Years, Adolescents and Young Adults Age 15-39 Years, and Older Adults Age 40+ Years, CBTRUS Statistical Report: NVSS 2013-2017

  • The most common causes of death in persons age 0-14 years was perinatal conditions (18.96 per 100,000).

  • Malignant brain and other CNS tumors among persons age 0-14 years had an AAAMR of 0.71 per 100,000 and were the most common cause of death in this age group, and the most common cause of cancer death.

  • Childhood brain and other CNS cancer, while rare, contributes substantially to cancer related mortality in this population, surpassing other cancers as the top reason for cancer mortality in those age 0-14 at diagnosis.

  • Accidents and adverse effects were the leading causes of death in persons age 15-39 years (39.58 per 100,000).

  • Malignant brain and other CNS tumors among persons age 15-39 years had an AAAMR of 0.96 per 100,000 and were the 12th most common cause of death in this age group and the 5th most common cause of cancer death, where their AAAMR was similar to that of leukemia.

  • Heart disease was the largest contributor to mortality in persons age 40+ years in the US, with an AAAMR of 381.28 per 100,000 for major cardiovascular diseases.

  • Malignant brain and other CNS tumors among persons age 40+ years had an AAAMR of 9.12 per 100,000 and were the 26th most common cause of death and the 3rd most common cause of cancer death.

Distributions and Incidence by Site, Behavior, Histology, and Year

Counts and rates from the 415,411 incident brain and other CNS tumors (123,484 malignant; 291,927 non-malignant shown in Fig. 5) reported during 2013-2017 by histology and demographic characteristics for all ages are presented in Table 3. Counts and rates are presented by histology and behaviors for selected histologies where there are a sufficient number of cases to calculate rates. The predominant tumor categories by behavior are presented in Supplementary Figures 3.

Fig. 5.

Fig. 5

Distributiona of Primary Brain and Other CNS Tumors by Behavior (Five-Year Total=415,411; Annual Average Cases=83,082), CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Table 3.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Behavior, and Sex, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology Total Male Female
5 year total Annual average % of all tumors Median Age Rate 95% CI 5 year total Annual average % Malignant % Non-Malignant Rate 95% CI 5 year total Annual average % Malignant % Non-Malignant Rate 95% CI
Tumors of Neuroepithelial Tissue 113,056 22,611 27.2% 57 6.56 6.52-6.60 63,606 12,721 92.8% 7.2% 7.71 7.65-7.77 49,450 9,890 92.3% 7.7% 5.55 5.50-5.60
Pilocytic Astrocytoma 5,167 1,033 1.2% 12 0.35 0.34-0.36 2,681 536 100.0% 0.0% 0.36 0.35-0.37 2,486 497 100.0% 0.0% 0.34 0.33-0.36
Diffuse Astrocytoma 7,428 1,486 1.8% 46 0.45 0.44-0.46 4,162 832 100.0% 0.0% 0.52 0.50-0.54 3,266 653 100.0% 0.0% 0.39 0.38-0.40
Anaplastic Astrocytoma 7,116 1,423 1.7% 53 0.42 0.41-0.43 3,892 778 100.0% 0.0% 0.48 0.46-0.49 3,224 645 100.0% 0.0% 0.37 0.36-0.38
Unique Astrocytoma Variants 1,123 225 0.3% 23 0.07 0.07-0.08 611 122 68.9% 31.1% 0.08 0.07-0.09 512 102 68.0% 32.0% 0.07 0.06-0.07
Malignant 769 154 -- 32 0.05 0.04-0.05 421 84 -- -- 0.05 0.05-0.06 348 70 -- -- 0.04 0.04-0.05
Non-Malignant 354 71 -- 11 0.02 0.02-0.03 190 38 -- -- 0.03 0.02-0.03 164 33 -- -- 0.02 0.02-0.03
Glioblastoma 60,056 12,011 14.5% 65 3.23 3.20-3.25 34,793 6,959 100.0% 0.0% 4.03 3.98-4.07 25,263 5,053 100.0% 0.0% 2.54 2.50-2.57
Oligodendroglioma 3,698 740 0.9% 44 0.23 0.23-0.24 2,031 406 100.0% 0.0% 0.26 0.25-0.27 1,667 333 100.0% 0.0% 0.21 0.20-0.22
Anaplastic Oligodendroglioma 1,859 372 0.4% 49 0.11 0.11-0.12 1,036 207 99.9% 0.1% 0.13 0.12-0.14 823 165 100.0% 0.0% 0.10 0.09-0.11
Oligoastrocytic Tumors 1,572 314 0.4% 42 0.10 0.09-0.10 893 179 100.0% 0.0% 0.11 0.11-0.12 679 136 99.9% 0.1% 0.08 0.08-0.09
Ependymal Tumors 6,843 1,369 1.6% 45 0.42 0.41-0.43 3,911 782 55.6% 44.4% 0.49 0.47-0.50 2,932 586 61.4% 38.6% 0.36 0.35-0.37
Malignant 3,972 794 -- 23 0.25 0.24-0.26 2,173 435 -- -- 0.27 0.26-0.29 1,799 360 -- -- 0.22 0.21-0.24
Non-Malignant 2,871 574 -- 48 0.17 0.17-0.18 1,738 348 -- -- 0.21 0.20-0.22 1,133 227 -- -- 0.14 0.13-0.14
Glioma Malignant, NOS 8,093 1,619 1.9% 36 0.51 0.50-0.52 4,089 818 100.0% 0.0% 0.53 0.51-0.55 4,004 801 100.0% 0.0% 0.49 0.47-0.50
Choroid Plexus Tumors 827 165 0.2% 20 0.05 0.05-0.06 425 85 16.7% 83.3% 0.06 0.05-0.06 402 80 14.2% 85.8% 0.05 0.05-0.06
Malignant 128 26 -- 2 0.01 0.01-0.01 71 14 -- -- 0.01 0.01-0.01 57 11 -- -- 0.01 0.01-0.01
Non-Malignant 699 140 -- 24 0.05 0.04-0.05 354 71 -- -- 0.05 0.04-0.05 345 69 -- -- 0.04 0.04-0.05
Other Neuroepithelial Tumors 107 21 0.0% 34 0.01 0.01-0.01 40 8 50.0% 50.0% 0.01 0.00-0.01 67 13 68.7% 31.3% 0.01 0.01-0.01
Malignant 66 13 -- 26.5 0.00 0.00-0.01 20 4 -- -- 0.00 0.00-0.00 46 9 -- -- 0.01 0.00-0.01
Non-Malignant 41 8 -- 43 0.00 0.00-0.00 20 4 -- -- 0.00 0.00-0.00 21 4 -- -- 0.00 0.00-0.00
Neuronal and Mixed Neuronal Glial Tumors 4,934 987 1.2% 26 0.32 0.31-0.33 2,672 534 20.5% 79.5% 0.34 0.33-0.36 2,262 452 17.6% 82.4% 0.29 0.28-0.31
Malignant 947 189 -- 52 0.06 0.05-0.06 549 110 -- -- 0.07 0.06-0.07 398 80 -- -- 0.05 0.04-0.05
Non-Malignant 3,987 797 -- 22 0.26 0.25-0.27 2,123 425 -- -- 0.28 0.27-0.29 1,864 373 -- -- 0.25 0.24-0.26
Tumors of the Pineal Region 787 157 0.2% 34 0.05 0.05-0.05 329 66 69.6% 30.4% 0.04 0.04-0.05 458 92 47.6% 52.4% 0.06 0.05-0.06
Malignant 447 89 -- 27 0.03 0.03-0.03 229 46 -- -- 0.03 0.03-0.03 218 44 -- -- 0.03 0.02-0.03
Non-Malignant 340 68 -- 43 0.02 0.02-0.02 100 20 -- -- 0.01 0.01-0.02 240 48 -- -- 0.03 0.03-0.03
Embryonal Tumors 3,446 689 0.8% 9 0.23 0.23-0.24 2,041 408 98.0% 2.0% 0.27 0.26-0.29 1,405 281 95.9% 4.1% 0.19 0.18-0.20
Tumors of Cranial and Spinal Nerves 35,600 7,120 8.6% 57 2.03 2.01-2.05 17,038 3,408 0.7% 99.3% 2.02 1.99-2.06 18,562 3,712 0.6% 99.4% 2.05 2.02-2.08
Nerve Sheath Tumors 35,560 7,112 8.6% 57 2.03 2.01-2.05 17,013 3,403 0.7% 99.3% 2.02 1.99-2.05 18,547 3,709 0.6% 99.4% 2.04 2.01-2.07
Malignant 223 45 -- 54 0.01 0.01-0.02 112 22 -- -- 0.01 0.01-0.02 111 22 -- -- 0.01 0.01-0.02
Non-Malignant 35,337 7,067 -- 57 2.02 1.99-2.04 16,901 3,380 -- -- 2.01 1.98-2.04 18,436 3,687 -- -- 2.03 2.00-2.06
Other Tumors of Cranial and Spinal Nerves 40 8 0.0% 54.5 0.00 0.00-0.00 -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 163,619 32,724 39.4% 66 9.09 9.04-9.13 45,497 9,099 2.6% 97.4% 5.56 5.51-5.61 118,122 23,624 1.1% 98.9% 12.22 12.15-12.29
Meningioma 159,038 31,808 38.3% 66 8.81 8.77-8.86 43,082 8,616 1.8% 98.2% 5.26 5.21-5.31 115,956 23,191 0.8% 99.2% 11.96 11.89-12.03
Malignant 1,750 350 -- 65 0.10 0.09-0.10 768 154 -- -- 0.09 0.08-0.10 982 196 -- -- 0.10 0.09-0.11
Non-Malignant 157,288 31,458 -- 66 8.72 8.67-8.76 42,314 8,463 -- -- 5.17 5.12-5.22 114,974 22,995 -- -- 11.86 11.79-11.93
Mesenchymal Tumors 1,462 292 0.4% 49 0.09 0.08-0.09 722 144 34.9% 65.1% 0.09 0.08-0.10 740 148 30.8% 69.2% 0.09 0.08-0.09
Primary Melanocytic Lesions 108 22 0.0% 57.5 0.01 0.00-0.01 55 11 81.8% 18.2% 0.01 0.00-0.01 53 11 50.9% 49.1% 0.01 0.00-0.01
Other Neoplasms Related to the Meninges 3,011 602 0.7% 49 0.18 0.17-0.19 1,638 328 8.2% 91.8% 0.20 0.19-0.21 1,373 275 8.4% 91.6% 0.16 0.15-0.17
Lymphomas and Hematopoietic Neoplasms 8,150 1,630 2.0% 67 0.45 0.44-0.46 4,129 826 -- -- 0.49 0.48-0.51 4,021 804 -- -- 0.41 0.40-0.42
Lymphoma 7,919 1,584 1.9% 67 0.43 0.42-0.44 4,002 800 100.0% 0.0% 0.48 0.46-0.49 3,917 783 100.0% 0.0% 0.40 0.38-0.41
Other Hematopoietic Neoplasms 231 46 0.1% 47 0.01 0.01-0.02 127 25 96.1% 3.9% 0.02 0.01-0.02 104 21 92.3% 7.7% 0.01 0.01-0.02
Germ Cell Tumors and Cysts 1,585 317 0.4% 16 0.11 0.10-0.11 1,094 219 76.7% 23.3% 0.14 0.14-0.15 491 98 52.1% 47.9% 0.07 0.06-0.07
Germ Cell Tumors, Cysts and Heterotopias 1,585 317 0.4% 16 0.11 0.10-0.11 1,094 219 76.7% 23.3% 0.14 0.14-0.15 491 98 52.1% 47.9% 0.07 0.06-0.07
Malignant 1,095 219 -- 15 0.07 0.07-0.08 839 168 -- -- 0.11 0.10-0.12 256 51 -- -- 0.04 0.03-0.04
Non-Malignant 490 98 -- 25.5 0.03 0.03-0.03 255 51 -- -- 0.03 0.03-0.04 235 47 -- -- 0.03 0.03-0.03
Tumors of Sellar Region 73,340 14,668 17.7% 51 4.39 4.36-4.43 33,190 6,638 0.3% 99.7% 4.02 3.98-4.07 40,150 8,030 0.1% 99.9% 4.85 4.80-4.90
Tumors of the Pituitary 70,211 14,042 16.9% 51 4.20 4.17-4.23 31,611 6,322 0.3% 99.7% 3.82 3.78-3.87 38,600 7,720 0.1% 99.9% 4.66 4.61-4.71
Malignant 142 28 -- 57 0.01 0.01-0.01 86 17 -- -- 0.01 0.01-0.01 56 11 -- -- 0.01 0.00-0.01
Non-Malignant 70,069 14,014 -- 51 4.19 4.16-4.22 31,525 6,305 -- -- 3.81 3.77-3.86 38,544 7,709 -- -- 4.65 4.61-4.70
Craniopharyngioma 3,129 626 0.8% 44 0.19 0.19-0.20 1,579 316 0.6% 99.4% 0.20 0.19-0.21 1,550 310 0.3% 99.7% 0.19 0.18-0.20
Unclassified Tumors 20,061 4,012 4.8% 64 1.16 1.14-1.17 9,087 1,817 34.9% 65.1% 1.15 1.12-1.17 10,974 2,195 31.8% 68.2% 1.18 1.15-1.20
Hemangioma 5,731 1,146 1.4% 50 0.34 0.34-0.35 2,518 504 0.4% 99.6% 0.31 0.30-0.33 3,213 643 0.4% 99.6% 0.38 0.36-0.39
Neoplasm Unspecified 14,136 2,827 3.4% 70 0.80 0.79-0.82 6,451 1,290 48.6% 51.4% 0.82 0.80-0.84 7,685 1,537 44.9% 55.1% 0.79 0.77-0.81
Malignant 6,587 1,317 -- 76 0.36 0.35-0.37 3,135 627 -- -- 0.40 0.39-0.41 3,452 690 -- -- 0.33 0.32-0.35
Non-Malignant 7,549 1,510 -- 63 0.44 0.43-0.45 3,316 663 -- -- 0.42 0.41-0.44 4,233 847 -- -- 0.46 0.44-0.47
All Other 194 39 0.0% 66 0.01 0.01-0.01 118 24 20.3% 79.7% 0.02 0.01-0.02 76 15 31.6% 68.4% 0.01 0.01-0.01
TOTAL c 415,411 83,082 -- 60 23.79 23.71-23.86 173,641 34,728 39.5% 60.5% 21.09 20.99-21.20 241,770 48,354 22.7% 77.3% 26.31 26.21-26.42
Malignant 123,484 24,697 29.7% 60 7.08 7.04-7.12 68,578 13,716 -- -- 8.30 8.24-8.36 54,906 10,981 -- -- 6.01 5.95-6.06
Non-Malignant 291,927 58,385 70.3% 61 16.71 16.64-16.77 105,063 21,013 -- -- 12.80 12.72-12.88 186,864 37,373 -- -- 20.31 20.21-20.40

a. Annual average cases are calculated by dividing the five-year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Refers to all brain tumors including histologies not presented in this table.

-- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

Incidence by Year and Behavior

The overall annual age-adjusted incidence rates of all primary brain and other CNS tumors by year, 2013-2017, and behavior are presented in Supplementary Figure 4. The incidence rates for all primary brain and other CNS tumors, 2013-2017, did not differ substantially by year (both overall and by behavior). AAAIR stratified by sex are presented in Supplementary Figure 5.

Distribution of Tumors by Site and Histology

The distribution of all primary brain and other CNS tumors by site and histology is presented in Fig. 6. Distributions for malignant and non-malignant tumors are presented in Fig. 7 and Fig. 8, respectively. Distribution and incidence by histology is also presented in Table 3.

Fig. 6.

Fig. 6

Distributiona of All Primary Brain and Other CNS Tumors (Malignant and Non-Malignant Combined; Five-Year Total=415,411; Annual Average Cases=83,082), by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Fig. 7.

Fig. 7

Distributiona of Malignant Primary Brain and Other CNS Tumors (Five-Year Total=123,484; Annual Average Cases=24,697), by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Fig. 8.

Fig. 8

Distributiona of All Non-Malignant Primary Brain and Other CNS Tumors (Five-Year Total=291,927; Annual Average Cases=58,385), by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • Overall, the most common tumor site was the meninges, representing 38.4% of all tumors.

  • Frontal (7.9%), temporal (5.8%), parietal (3.4%), and occipital lobes (0.9%) accounted for 18% of all tumors.

  • The cranial nerves and the spinal cord/cauda equina accounted for 10.1% of all tumors.

  • The pituitary and craniopharyngeal duct accounted for 17.9% of all tumors.

  • The most frequently reported histology overall was meningioma (38.3%), followed by tumors of the pituitary (16.9%) and glioblastoma (14.5%).

  • Tumors of the pituitary and nerve sheath tumors combined accounted for slightly more than one-fourth of all tumors (25.5%), the vast majority of which were non-malignant.

  • For malignant tumors, frontal (24.3%), temporal (17.5%), parietal (10.4%), and occipital (2.6%) accounted for 54.8% of tumors (Fig. 7).

  • The most common of all malignant CNS tumors was glioblastoma (48.6%).

  • For non-malignant tumors, 53.9% of all tumors occurred in the meninges (Fig. 9C).

  • The most common histology among non-malignant tumors was meningioma (53.9%).

  • The most common non-malignant nerve sheath tumor (based on multiple sites in the brain and other CNS) was schwannoma (defined by histology code 9560). These tumors can occur in many sites (Supplementary Figure 6), but most commonly occur on the acoustic nerve, where they are called vestibular schwannoma (also formerly called acoustic neuromas) (74.7% of all nerve sheath tumors).

Fig. 9.

Fig. 9

Distributiona of Primary Brain and Other CNS Gliomasb (Five-Year Total=104,103; Annual Average Cases=20,821) by A) Site and B) Histology Subtypes, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Distribution of brain and other CNS tumors by site and histology for males and females are presented in Supplementary Figure 7 and Supplementary Figure 8, respectively.

Distribution of Gliomas by Site and Histology

The broad category glioma (ICD-O-3 histology codes 9380-9384, 9391-9460, see Table 2 for more information) represented approximately 25.1% of all primary brain and other CNS tumors and 80.8% of malignant tumors. The distribution of gliomas by site and histology are presented in Fig. 9A and Fig. 9B, respectively.

  • The majority of gliomas occurred in the supra-tentorium (frontal, temporal, parietal, and occipital lobes combined) (61.4%). Only a very small proportion of gliomas occurred in areas of the CNS other than the brain (i.e. the spinal cord).

  • Glioblastoma accounted for the majority of gliomas (57.7%).

Incidence Rates by Major Histology Grouping, Specific Histologies, and Behavior

Incidence rates overall by major histology grouping, specific histology, and behavior are presented in Table 3.

  • Among CBTRUS major histology groupings, incidence rates were highest for tumors of the meninges (9.09 per 100,000 population) followed by tumors of the neuroepithelial tissue (6.56 per 100,000 population), tumors of the sellar region (4.39 per 100,000 population), and tumors of the cranial and spinal nerves (2.03 per 100,000 population).

  • Among CBTRUS specific histology groupings, incidence rates were highest for meningiomas (8.81 per 100,000 population), tumors of the pituitary (4.20 per 100,000 population), glioblastomas (3.23 per 100,000 population), and nerve sheath tumors (2.03 per 100,000 population).

  • For malignant tumors, the incidence rate was highest for glioblastoma (3.23 per 100,000 population), followed by glioma malignant, NOS (0.51 per 100,000), diffuse astrocytoma (0.45 per 100,000 population) and lymphoma (0.43 per 100,000 population).

  • For non-malignant tumors, the incidence rate was highest for non-malignant meningioma (8.72 per 100,000 population), followed by non-malignant tumors of the pituitary (4.19 per 100,000 population).

Incidence rates for selection non-malignant histologies overall, by sex, age groups, race, and ethnicity are presented in Table 4, including vestibular schwannoma, pituitary adenoma, WHO grade I meningioma, and WHO grade II meningioma. These histologies are subsets of histologies presented in the overall CBTRUS histology grouping scheme (nerve sheath tumors, tumors of the pituitary, and meningioma) but are presented here due to particular clinical interest in these subgroups.

Table 4.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Selected Non-Malignant Histologies by Sex, Age Groups, Race, and Hispanic Ethnicity, Histology, and Age at Diagnosis, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Group Vestibular Schwannomac Pituitary Adenomad WHO Grade I Meningiomae WHO Grade II Meningiomaf
5-Year total Annual Average Rate 95% CI 5-Year total Annual Average Rate 95% CI 5-Year total Annual Average Rate 95% CI 5-Year total Annual Average Rate 95% CI
Sex
Male 12,349 2,470 1.45 1.42-1.48 27,424 5,485 3.31 3.27-3.35 39,166 7,833 4.79 4.74-4.84 3,148 630 0.38 0.36-0.39
Female 14,039 2,808 1.53 1.50-1.55 33,206 6,641 3.99 3.95-4.04 110,469 22,094 11.37 11.31-11.44 4,505 901 0.49 0.47-0.50
Age Groups
0-14 Years 197 39 0.07 0.06-0.08 861 172 0.29 0.27-0.31 202 40 0.07 0.06-0.08 82 16 0.03 0.02-0.03
15-39 Years 3,584 717 0.70 0.67-0.72 17,234 3,447 3.27 3.22-3.32 8,449 1,690 1.69 1.66-1.73 867 173 0.17 0.16-0.18
40-64 Years 14,168 2,834 2.56 2.52-2.61 26,299 5,260 5.00 4.94-5.06 58,789 11,758 10.45 10.36-10.54 3,504 701 0.63 0.61-0.66
65+ Years 8,439 1,688 3.53 3.45-3.60 16,236 3,247 6.92 6.81-7.02 82,195 16,439 35.57 35.32-35.81 3,200 640 1.37 1.33-1.42
Race
White 22,745 4,549 1.58 1.56-1.60 43,492 8,698 3.25 3.22-3.29 121,777 24,355 8.11 8.06-8.15 5,969 1,194 0.41 0.40-0.42
Black 1,449 290 0.68 0.65-0.72 12,120 2,424 5.82 5.72-5.93 18,689 3,738 9.54 9.40-9.68 1,131 226 0.55 0.52-0.59
American Indian/Alaska Native 154 31 0.75 0.63-0.89 502 100 2.49 2.26-2.73 890 178 5.29 4.93-5.67 37 7 0.23 0.15-0.32
Asian or Pacific Islander 1,174 235 1.18 1.11-1.25 2,454 491 2.49 2.39-2.60 5,270 1,054 5.87 5.71-6.03 357 71 0.37 0.33-0.41
Hispanic Ethnicity
Non-Hispanic 24,181 4,836 1.57 1.55-1.59 50,948 10,190 3.55 3.51-3.58 135,219 27,044 8.39 8.34-8.43 6,976 1,395 0.45 0.44-0.46
Hispanic 2,207 441 1.00 0.96-1.05 9,682 1,936 4.08 3.99-4.17 14,416 2,883 7.79 7.66-7.92 677 135 0.33 0.30-0.35
TOTAL 21,453 4,291 1.20 1.19-1.22 60,630 12,126 3.61 3.58-3.64 149,635 29,927 8.29 8.24-8.33 7,653 1,531 0.43 0.42-0.44

a. Annual average cases are calculated by dividing the five year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. ICD-O-3 histology code 9560/0 and ICD-O-3 topography code C72.4 and C72.5.

d. ICD-O-3 histology code 8272/0 and ICD-O-3 topography code C75.1.

e. ICD-O-3 histology codes 9530/0, 9531/0, 9532/0, 9533/0, 9534/0, and 9537/0.

f. ICD-O-3 histology codes 9530/1, 9531/1, 9532/1, 9533/1, 9534/1, 9537/1, 9538/1, and 9539/1.

- Counts and rates are not presented when fewer than 16 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval

Distributions and Incidence by Age

Incidence Rates by Age

The overall AAAIR for 2013-2017 for all primary brain and other CNS tumors was 23.79 per 100,000 population (Table 3). The overall incidence rate was 5.83 per 100,000 population for children age 0-14 years, 11.54 per 100,000 population for adolescents and young adults age 15-39 years, and 42.85 per 100,000 population for adults age 40+ years (Table 5). The overall incidence rates of tumors by behavior and age group (age 0-14 years, 0-19 years and 20+ years) are presented in Fig. 10. AAAIR stratified by sex are presented in Supplementary Figure 9.

Table 5.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals of Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and NCI Age Group, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology Age at Diagnosis
Childrenc (0-14) Years AYAd (15-39) Years Adults (40+) Years
5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI
Tumors of Neuroepithelial Tissue 12,719 2,544 4.19 4.12-4.26 18,350 3,670 3.46 3.41-3.52 81,987 16,397 10.30 10.23-10.37
Pilocytic Astrocytoma 3,128 626 1.03 0.99-1.07 1,460 292 0.27 0.26-0.29 579 116 0.08 0.07-0.09
Diffuse Astrocytoma 703 141 0.23 0.21-0.25 2,392 478 0.45 0.43-0.47 4,333 867 0.57 0.55-0.58
Anaplastic Astrocytoma 283 57 0.09 0.08-0.11 1,858 372 0.35 0.34-0.37 4,975 995 0.64 0.62-0.66
Unique Astrocytoma Variants 379 76 0.13 0.11-0.14 387 77 0.07 0.06-0.08 357 71 0.05 0.04-0.05
Malignant 158 32 0.05 0.04-0.06 289 58 0.05 0.05-0.06 322 64 0.04 0.04-0.05
Non-Malignant 221 44 0.07 0.06-0.08 98 20 0.02 0.01-0.02 35 7 0.00 0.00-0.01
Glioblastoma 480 96 0.16 0.14-0.17 2,767 553 0.54 0.52-0.56 56,809 11,362 6.97 6.91-7.03
Oligodendroglioma 86 17 0.03 0.02-0.04 1,431 286 0.27 0.26-0.29 2,181 436 0.30 0.29-0.32
Anaplastic Oligodendroglioma -- -- -- -- -- -- -- -- 1,346 269 0.18 0.17-0.19
Oligoastrocytic Tumors 38 8 0.01 0.01-0.02 643 129 0.12 0.11-0.13 891 178 0.12 0.11-0.13
Ependymal Tumors 959 192 0.32 0.30-0.34 1,893 379 0.36 0.34-0.38 3,991 798 0.53 0.51-0.55
Malignant 845 169 0.28 0.26-0.30 1,033 207 0.20 0.18-0.21 2,094 419 0.28 0.26-0.29
Non-Malignant 114 23 0.04 0.03-0.05 860 172 0.16 0.15-0.17 1,897 379 0.25 0.24-0.26
Glioma Malignant, NOS 2,558 512 0.84 0.81-0.88 1,662 332 0.31 0.30-0.33 3,873 775 0.50 0.49-0.52
Choroid Plexus Tumors 365 73 0.12 0.11-0.13 216 43 0.04 0.04-0.05 246 49 0.03 0.03-0.04
Malignant 101 20 0.03 0.03-0.04 -- -- -- -- -- -- -- --
Non-Malignant 264 53 0.09 0.08-0.10 -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 25 5 0.01 0.01-0.01 36 7 0.01 0.00-0.01 46 9 0.01 0.00-0.01
Malignant -- -- -- -- -- -- -- -- 22 4 0.00 0.00-0.00
Non-Malignant -- -- -- -- -- -- -- -- 24 5 0.00 0.00-0.00
Neuronal and Mixed Neuronal Glial Tumors 1,298 260 0.43 0.41-0.45 1,981 396 0.37 0.35-0.38 1,655 331 0.22 0.21-0.23
Malignant 70 14 0.02 0.02-0.03 199 40 0.04 0.03-0.04 678 136 0.09 0.08-0.09
Non-Malignant 1,228 246 0.41 0.38-0.43 1,782 356 0.33 0.32-0.35 977 195 0.14 0.13-0.14
Tumors of the Pineal Region 153 31 0.05 0.04-0.06 289 58 0.05 0.05-0.06 345 69 0.05 0.04-0.05
Malignant 133 27 0.04 0.04-0.05 162 32 0.03 0.03-0.03 152 30 0.02 0.02-0.02
Non-Malignant 20 4 0.01 0.00-0.01 127 25 0.02 0.02-0.03 193 39 0.03 0.02-0.03
Embryonal Tumors 2,252 450 0.74 0.71-0.77 834 167 0.15 0.14-0.16 360 72 0.05 0.04-0.06
Medulloblastoma 1,463 293 0.48 0.46-0.51 632 126 0.12 0.11-0.13 155 31 0.02 0.02-0.03
Primitive neuroectodermal tumors 214 43 0.07 0.06-0.08 102 20 0.02 0.02-0.02 111 22 0.01 0.01-0.02
Atypical teratoid rhabdoid tumor 374 75 0.12 0.11-0.14 28 6 0.01 0.00-0.01 -- -- -- --
Other embryonal histologies 201 40 0.07 0.06-0.08 72 14 0.01 0.01-0.02 81 16 0.01 0.01-0.01
Tumors of Cranial and Spinal Nerves 840 168 0.28 0.26-0.30 5,430 1,086 1.05 1.02-1.08 29,330 5,866 3.72 3.67-3.76
Nerve Sheath Tumors 840 168 0.28 0.26-0.30 5,422 1,084 1.05 1.02-1.08 29,298 5,860 3.71 3.67-3.76
Malignant -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- --
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- 32 6 0.00 0.00-0.01
Tumors of Meninges 592 118 0.20 0.18-0.21 10,765 2,153 2.14 2.09-2.18 152,262 30,452 19.26 19.16-19.36
Meningioma 306 61 0.10 0.09-0.11 9,438 1,888 1.89 1.85-1.92 149,294 29,859 18.88 18.78-18.97
Malignant 22 4 0.01 0.00-0.01 122 24 0.02 0.02-0.03 1,606 321 0.20 0.19-0.21
Non-Malignant 284 57 0.09 0.08-0.11 9,316 1,863 1.86 1.82-1.90 147,688 29,538 18.68 18.58-18.77
Mesenchymal Tumors 223 45 0.07 0.06-0.08 334 67 0.06 0.06-0.07 905 181 0.12 0.11-0.13
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- 85 17 0.01 0.01-0.01
Other Neoplasms Related to the Meninges 55 11 0.02 0.01-0.02 978 196 0.18 0.17-0.20 1,978 396 0.26 0.25-0.27
Lymphoma and Hematopoietic Neoplasms 78 16 0.03 0.02-0.03 548 110 0.11 0.10-0.11 7,524 1,505 0.94 0.92-0.96
Lymphoma 29 6 0.01 0.01-0.01 491 98 0.09 0.09-0.10 7,399 1,480 0.93 0.90-0.95
Other Hematopoietic Neoplasms 49 10 0.02 0.01-0.02 57 11 0.01 0.01-0.01 125 25 0.02 0.01-0.02
Germ Cell Tumors and Cysts 694 139 0.23 0.21-0.25 700 140 0.13 0.12-0.14 191 38 0.03 0.02-0.03
Germ Cell Tumors, Cysts and Heterotopias 694 139 0.23 0.21-0.25 700 140 0.13 0.12-0.14 191 38 0.03 0.02-0.03
Malignant 508 102 0.17 0.15-0.18 558 112 0.10 0.09-0.11 29 6 0.00 0.00-0.01
Non-Malignant 186 37 0.06 0.05-0.07 142 28 0.03 0.02-0.03 162 32 0.02 0.02-0.03
Tumors of Sellar Region 1,715 343 0.57 0.54-0.60 21,491 4,298 4.07 4.02-4.13 50,134 10,027 6.57 6.51-6.63
Tumors of the Pituitary 1,029 206 0.34 0.32-0.36 20,782 4,156 3.94 3.88-3.99 48,400 9,680 6.34 6.28-6.40
Malignant -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- --
Craniopharyngioma 686 137 0.23 0.21-0.24 709 142 0.13 0.12-0.14 1,734 347 0.22 0.21-0.24
Unclassified Tumors 1,035 207 0.34 0.32-0.36 3,074 615 0.58 0.56-0.60 15,952 3,190 2.04 2.01-2.07
Hemangioma 374 75 0.12 0.11-0.14 1,506 301 0.29 0.27-0.30 3,851 770 0.50 0.49-0.52
Neoplasm Unspecified 628 126 0.21 0.19-0.22 1,548 310 0.29 0.28-0.31 11,960 2,392 1.52 1.49-1.55
Malignant 164 33 0.05 0.05-0.06 348 70 0.07 0.06-0.07 6,075 1,215 0.76 0.74-0.78
Non-Malignant 464 93 0.15 0.14-0.17 1,200 240 0.23 0.21-0.24 5,885 1,177 0.76 0.74-0.78
All Other 33 7 0.01 0.01-0.02 20 4 0.00 0.00-0.01 141 28 0.02 0.01-0.02
TOTAL e 17,673 3,535 5.83 5.74-5.91 60,358 12,072 11.54 11.45-11.63 337,380 67,476 42.85 42.71-43.00
Malignant 11,738 2,348 3.87 3.80-3.94 17,073 3,415 3.23 3.18-3.28 94,673 18,935 11.86 11.78-11.94
Non-Malignant 5,935 1,187 1.96 1.91-2.01 43,285 8,657 8.31 8.23-8.39 242,707 48,541 30.99 30.87-31.12

a. Annual average cases are calculated by dividing the five-year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Children as defined by the National Cancer Institute, see: http://www.cancer.gov/researchandfunding/snapshots/pediatric.

d. Adolescents and Young Adults (AYA), as defined by the National Cancer Institute, see: http://www.cancer.gov/cancertopics/aya.

e. Refers to all brain tumors including histologies not presented in this table.

-- Counts and rates are not presented when fewer than 16 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: AYA, Adolescents and Young Adults; CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

Fig. 10.

Fig. 10

Average Annual Age-Adjusted Incidence Ratesa of Primary Brain and Other CNS Tumors by Age and Behavior, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Incidence Rates by Age and Histology

The age-adjusted incidence rates by age and histology at diagnosis are presented in Tables 5-7 as well as in Fig. 11A (Age 0-19 Years), and Fig. 11B (Age 20+ Years).

Table 7.

Five-Year Total, Average Annual Age-Adjusted and Age-Specific Incidence Ratesa with 95% Confidence Intervals for Adults (Age 20+ Years), Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and Age at Diagnosis, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology Age At Diagnosis
20-34 years 35-44 years 45-54 years 55-64 years 65-74 years 75-84 years 85+ years
Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI
Tumors of Neuroepithelial Tissue 3.34 3.27-3.40 4.47 4.37-4.56 6.71 6.61-6.82 11.30 11.16-11.45 16.79 16.57-17.01 19.26 18.93-19.59 12.03 11.65-12.42
Pilocytic Astrocytoma 0.21 0.20-0.23 0.13 0.11-0.14 0.09 0.08-0.11 0.07 0.06-0.08 0.07 0.05-0.08 0.05 0.04-0.07 -- --
Diffuse Astrocytoma 0.49 0.46-0.51 0.50 0.46-0.53 0.47 0.44-0.50 0.56 0.53-0.59 0.77 0.72-0.81 0.82 0.75-0.89 0.47 0.40-0.55
Anaplastic Astrocytoma 0.38 0.36-0.40 0.50 0.47-0.53 0.51 0.48-0.54 0.68 0.65-0.72 0.90 0.85-0.95 0.93 0.86-1.00 0.41 0.34-0.49
Unique Astrocytoma Variants 0.07 0.06-0.08 0.04 0.03-0.05 0.03 0.03-0.04 0.04 0.03-0.05 0.06 0.05-0.08 0.08 0.06-0.11 0.07 0.04-0.11
Malignant 0.05 0.04-0.06 0.04 0.03-0.04 0.03 0.02-0.03 0.04 0.03-0.05 0.06 0.05-0.07 0.08 0.06-0.11 0.07 0.04-0.10
Non-Malignant 0.02 0.01-0.02 0.01 0.00-0.01 0.01 0.00-0.01 -- -- -- -- -- -- -- --
Glioblastoma 0.47 0.45-0.49 1.27 1.22-1.32 3.60 3.53-3.69 8.06 7.94-8.18 13.00 12.81-13.20 15.30 15.01-15.59 9.01 8.68-9.35
Oligodendroglioma 0.28 0.26-0.30 0.43 0.40-0.46 0.38 0.36-0.41 0.27 0.25-0.30 0.19 0.17-0.22 0.14 0.11-0.17 0.06 0.04-0.10
Anaplastic Oligodendroglioma 0.10 0.09-0.11 0.18 0.17-0.20 0.21 0.19-0.23 0.19 0.18-0.21 0.16 0.14-0.18 0.12 0.09-0.14 -- --
Oligoastrocytic Tumors 0.14 0.12-0.15 0.17 0.15-0.19 0.14 0.12-0.15 0.11 0.10-0.13 0.11 0.09-0.13 0.07 0.05-0.09 -- --
Ependymal Tumors 0.35 0.33-0.37 0.52 0.49-0.55 0.55 0.52-0.59 0.56 0.53-0.60 0.60 0.56-0.64 0.40 0.36-0.45 0.12 0.08-0.16
Malignant 0.18 0.17-0.20 0.27 0.25-0.29 0.29 0.27-0.31 0.30 0.28-0.32 0.32 0.29-0.35 0.23 0.19-0.26 0.06 0.04-0.10
Non-Malignant 0.17 0.15-0.18 0.25 0.23-0.27 0.27 0.25-0.29 0.26 0.24-0.29 0.28 0.25-0.31 0.18 0.15-0.21 0.05 0.03-0.09
Glioma Malignant, NOS 0.29 0.27-0.31 0.28 0.25-0.30 0.33 0.31-0.36 0.39 0.37-0.42 0.60 0.56-0.64 1.08 1.00-1.16 1.70 1.56-1.86
Choroid Plexus Tumors 0.04 0.03-0.05 0.04 0.03-0.05 0.03 0.02-0.03 0.04 0.03-0.05 0.03 0.02-0.04 0.04 0.03-0.06 -- --
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 0.01 0.00-0.01 0.01 0.00-0.01 -- -- -- -- -- -- -- -- -- --
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 0.33 0.31-0.35 0.26 0.24-0.28 0.25 0.23-0.27 0.22 0.20-0.24 0.21 0.19-0.24 0.16 0.14-0.20 0.08 0.05-0.12
Malignant 0.03 0.03-0.04 0.06 0.05-0.07 0.08 0.07-0.10 0.10 0.08-0.11 0.11 0.09-0.13 0.10 0.07-0.12 0.07 0.04-0.10
Non-Malignant 0.29 0.27-0.31 0.20 0.18-0.22 0.17 0.15-0.18 0.12 0.11-0.14 0.10 0.09-0.12 0.07 0.05-0.09 -- --
Tumors of the Pineal Region 0.06 0.05-0.06 0.05 0.04-0.06 0.05 0.04-0.06 0.04 0.04-0.05 0.05 0.04-0.06 0.02 0.01-0.04 -- --
Malignant 0.03 0.03-0.04 0.02 0.02-0.03 0.03 0.02-0.03 0.02 0.01-0.03 0.02 0.01-0.03 -- -- -- --
Non-Malignant 0.03 0.02-0.03 0.03 0.02-0.04 0.03 0.02-0.03 0.02 0.02-0.03 0.03 0.02-0.04 -- -- -- --
Embryonal Tumors 0.15 0.14-0.16 0.09 0.08-0.10 0.05 0.04-0.06 0.05 0.04-0.06 0.03 0.02-0.04 0.04 0.03-0.06 -- --
Tumors of Cranial and Spinal Nerves 0.95 0.91-0.98 2.15 2.08-2.21 3.27 3.19-3.35 4.49 4.40-4.58 5.33 5.20-5.45 4.20 4.04-4.35 1.85 1.70-2.00
Nerve Sheath Tumors 0.94 0.91-0.98 2.14 2.08-2.21 3.27 3.19-3.34 4.49 4.39-4.58 5.32 5.20-5.44 4.19 4.04-4.35 1.85 1.70-2.00
Malignant 0.01 0.01-0.01 0.02 0.01-0.02 0.02 0.01-0.03 0.02 0.01-0.02 0.04 0.03-0.05 0.02 0.01-0.04 -- --
Non-Malignant 0.94 0.90-0.97 2.13 2.06-2.19 3.25 3.17-3.32 4.47 4.38-4.56 5.28 5.16-5.41 4.17 4.02-4.33 1.82 1.67-1.98
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 1.71 1.67-1.76 5.81 5.70-5.91 10.92 10.78-11.06 16.93 16.75-17.11 29.54 29.25-29.84 43.77 43.28-44.26 55.23 54.41-56.06
Meningioma 1.46 1.42-1.50 5.49 5.39-5.60 10.55 10.41-10.68 16.51 16.33-16.68 29.08 28.79-29.37 43.33 42.84-43.82 55.08 54.26-55.91
Malignant 0.02 0.02-0.03 0.05 0.04-0.06 0.10 0.09-0.12 0.21 0.19-0.23 0.33 0.30-0.36 0.44 0.39-0.49 0.49 0.42-0.57
Non-Malignant 1.44 1.40-1.48 5.44 5.34-5.54 10.45 10.31-10.58 16.30 16.12-16.47 28.75 28.46-29.04 42.89 42.41-43.38 54.59 53.77-55.42
Mesenchymal Tumors 0.06 0.05-0.07 0.08 0.07-0.10 0.12 0.10-0.13 0.13 0.11-0.14 0.14 0.12-0.16 0.15 0.12-0.18 0.07 0.04-0.10
Primary Melanocytic Lesions -- -- -- -- 0.01 0.00-0.01 0.02 0.01-0.02 0.01 0.01-0.02 -- -- -- --
Other Neoplasms Related to the Meninges 0.19 0.17-0.20 0.22 0.20-0.25 0.25 0.23-0.27 0.28 0.26-0.31 0.31 0.28-0.34 0.27 0.23-0.31 0.07 0.05-0.11
Lymphomas and Hematopoietic Neoplasms 0.10 0.09-0.11 0.21 0.19-0.23 0.41 0.38-0.43 0.89 0.85-0.93 1.87 1.80-1.95 2.43 2.31-2.55 1.26 1.14-1.39
Lymphoma 0.09 0.08-0.10 0.20 0.18-0.22 0.39 0.37-0.42 0.88 0.83-0.92 1.85 1.77-1.92 2.41 2.29-2.53 1.24 1.12-1.37
Other Hematopoietic Neoplasms 0.01 0.01-0.01 0.01 0.01-0.02 0.01 0.01-0.02 0.02 0.01-0.02 0.03 0.02-0.04 -- -- -- --
Germ Cell Tumors and Cysts 0.11 0.10-0.12 0.03 0.03-0.04 0.03 0.02-0.03 0.02 0.02-0.03 0.02 0.02-0.03 -- -- -- --
Germ Cell Tumors, Cysts and Heterotopias 0.11 0.10-0.12 0.03 0.03-0.04 0.03 0.02-0.03 0.02 0.02-0.03 0.02 0.02-0.03 -- -- -- --
Malignant 0.08 0.07-0.09 0.01 0.00-0.01 -- -- -- -- -- -- -- -- -- --
Non-Malignant 0.03 0.02-0.03 0.03 0.02-0.03 0.02 0.02-0.03 0.02 0.01-0.03 0.02 0.01-0.03 -- -- -- --
Tumors of Sellar Region 4.05 3.99-4.12 5.64 5.53-5.74 5.71 5.61-5.81 6.58 6.47-6.69 8.35 8.19-8.50 8.54 8.33-8.76 5.31 5.06-5.58
Tumors of the Pituitary 3.94 3.87-4.01 5.46 5.36-5.56 5.50 5.40-5.60 6.32 6.21-6.43 8.07 7.92-8.22 8.33 8.11-8.54 5.23 4.98-5.49
Malignant -- -- 0.01 0.01-0.02 0.01 0.01-0.02 0.02 0.01-0.03 0.02 0.01-0.02 0.02 0.01-0.04 -- --
Non-Malignant 3.94 3.87-4.01 5.45 5.35-5.55 5.48 5.38-5.58 6.30 6.19-6.41 8.05 7.90-8.21 8.30 8.09-8.52 5.23 4.98-5.49
Craniopharyngioma 0.11 0.10-0.13 0.18 0.16-0.20 0.21 0.19-0.23 0.26 0.24-0.29 0.28 0.25-0.31 0.22 0.18-0.25 0.08 0.05-0.12
Unclassified Tumors 0.56 0.53-0.59 0.82 0.78-0.86 1.07 1.02-1.11 1.46 1.41-1.51 2.39 2.31-2.47 4.74 4.58-4.91 10.56 10.20-10.93
Hemangioma 0.28 0.26-0.30 0.40 0.37-0.43 0.45 0.42-0.48 0.51 0.48-0.55 0.59 0.55-0.63 0.66 0.60-0.72 0.49 0.42-0.58
Neoplasm Unspecified 0.28 0.26-0.30 0.42 0.39-0.45 0.61 0.58-0.65 0.93 0.89-0.98 1.77 1.70-1.84 4.03 3.89-4.18 9.98 9.63-10.34
Malignant 0.06 0.05-0.07 0.11 0.09-0.12 0.20 0.18-0.22 0.42 0.40-0.45 0.88 0.83-0.93 2.29 2.18-2.40 6.05 5.78-6.33
Non-Malignant 0.22 0.20-0.23 0.31 0.29-0.34 0.41 0.38-0.44 0.51 0.48-0.54 0.89 0.84-0.94 1.75 1.65-1.85 3.93 3.71-4.15
All Other -- -- -- -- -- -- 0.01 0.01-0.01 0.03 0.02-0.04 0.05 0.04-0.07 0.09 0.06-0.13
TOTAL b 10.82 10.70-10.93 19.12 18.93-19.32 28.11 27.89-28.34 41.68 41.40-41.96 64.29 63.86-64.73 82.96 82.28-83.64 86.27 85.24-87.31
Malignant 3.10 3.04-3.16 4.39 4.30-4.48 7.03 6.91-7.14 12.49 12.34-12.65 19.56 19.33-19.80 24.25 23.88-24.62 19.81 19.32-20.31
Non-Malignant 7.72 7.62-7.81 14.74 14.57-14.90 21.08 20.89-21.28 29.19 28.95-29.42 44.73 44.37-45.09 58.71 58.14-59.28 66.46 65.56-67.38

a. Rates are per 100,000 and age-adjusted to the 2000 US standard population.

b. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

Fig. 11.

Fig. 11

Age-Adjusted Incidence Ratesa of Brain and Other CNS Tumors by Selected Histologies and Age Group a) Age 0-19 Yearsb, B) Age 20+ Yearsb and CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • The incidence rate for all brain and other CNS tumors was highest among age 85+ years (86.27 per 100,000 population) and lowest among children and adolescents age 0-19 years (6.14 per 100,000 population).

  • Incidence rates of pilocytic astrocytoma, germ cell tumors, and embryonal tumors were higher in the younger age groups and lower with in older age group.

  • Incidence rates of meningioma increased with age.

  • Incidence rates declined with increasing age for those ages 0-19 years, particularly for the gliomas and embryonal tumors (primitive neuroectodermal tumor (PNET) and medulloblastoma).

Median Age at Diagnosis

The median age for all primary brain and other CNS tumors by histology is presented in Table 3. The overall median age at diagnosis was 60 years.

  • The histology-specific median ages ranged from 9 years for Embryonal Tumors to 70 years for Neoplasm Unspecified.

  • Pilocytic astrocytoma, choroid plexus tumors, neuronal and mixed neuronal-glial tumors, tumors of the pineal region, embryonal tumors, and germ cell tumors and cysts were histologies with younger median ages at diagnosis compared to other histologies.

  • Meningioma and glioblastoma were primarily diagnosed at older ages (median age of 66 and 65 years, respectively).

Distributions and Incidence by Sex

Distribution by Sex and Behavior

  • Overall, 42% of all tumors diagnosed between 2013 and 2017 occurred in males (173,641 tumors) and 58% in females (241,770 tumors) (Table 3).

  • Approximately 56% of the malignant tumors occurred in males (68,578 tumors between 2013 and 2017) and 44% in females (54,906 tumors between 2013 and 2017).

  • Approximately 36% of the non-malignant tumors occurred in males (105,063 tumors between 2013 and 2017) and 64% in females (186,864 tumors between 2013 and 2017).

Incidence Rates by Site and Sex

Incidence counts and average annual age-adjusted rates for brain and other CNS tumors by site and sex are presented in Table 8.

Table 8.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals of Brain and Other Central Nervous System Tumors by Sitec and Sex, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Site (ICD-O-3 Code) Total Male Female
5-Year total Annual Average % of All Tumors Rate 95% CI 5-Year total Annual Average % Malignant % Non-Malignant Rate 95% CI 5-Year total Annual Average % Malignant % Non-Malignant Rate 95% CI
Frontal, temporal, parietal, and occipital lobes of the brain (C71.1-C71.4) 74,890 14,978 15.3% 4.23 4.20-4.26 41,746 8,349 91.7% 8.3% 4.99 4.94-5.04 33,144 6,629 88.5% 11.5% 3.57 3.53-3.61
Frontal lobe (C71.1) 32,856 6,571 7.9% 1.88 1.86-1.90 17,467 3,493 92.3% 7.7% 2.11 2.07-2.14 15,389 3,078 89.9% 10.1% 1.68 1.65-1.71
Temporal lobe (C71.2) 24,233 4,847 5.8% 1.36 1.34-1.38 14,447 2,889 90.8% 9.2% 1.72 1.69-1.74 9,786 1,957 86.8% 13.2% 1.05 1.03-1.07
Parietal lobe (C71.3) 13,987 2,797 3.4% 0.78 0.76-0.79 7,706 1,541 93.4% 6.6% 0.91 0.89-0.93 6,281 1,256 89.1% 10.9% 0.66 0.64-0.68
Occipital lobe (C71.4) 3,814 763 0.9% 0.21 0.21-0.22 2,126 425 86.4% 13.6% 0.26 0.24-0.27 1,688 338 83.8% 16.2% 0.18 0.17-0.19
Cerebrum (C71.0) 7,213 1,443 1.7% 0.42 0.41-0.43 3,872 774 83.2% 16.8% 0.47 0.46-0.49 3,341 668 79.5% 20.5% 0.38 0.37-0.39
Ventricle (C71.5) 4,097 819 1.0% 0.26 0.25-0.26 2,218 444 42.1% 57.9% 0.28 0.27-0.29 1,879 376 40.1% 59.9% 0.23 0.22-0.24
Cerebellum (C71.6) 8,892 1,778 2.1% 0.56 0.55-0.57 4,737 947 63.9% 36.1% 0.61 0.59-0.63 4,155 831 55.6% 44.4% 0.52 0.50-0.53
Brain stem (C71.7) 6,174 1,235 1.5% 0.40 0.39-0.41 3,346 669 76.1% 23.9% 0.43 0.42-0.45 2,828 566 77.4% 22.6% 0.37 0.35-0.38
Other brain (C71.8-C71.9) 33,498 6,700 8.1% 1.90 1.88-1.92 17,687 3,537 83.8% 16.2% 2.16 2.12-2.19 15,811 3,162 78.9% 21.1% 1.68 1.65-1.70
Spinal cord and cauda equina (C72.0-C72.1) 12,905 2,581 3.1% 0.77 0.76-0.79 6,782 1,356 27.6% 72.4% 0.84 0.82-0.86 6,123 1,225 26.4% 73.6% 0.71 0.70-0.73
Cranial nerves (C72.2-C72.5) 28,850 5,770 6.9% 1.65 1.63-1.67 13,501 2,700 5.7% 94.3% 1.60 1.57-1.63 15,349 3,070 5.5% 94.5% 1.70 1.67-1.72
Other nervous system (C72.8-C72.9) 2,548 510 0.6% 0.15 0.14-0.15 1,295 259 56.8% 43.2% 0.16 0.15-0.17 1,253 251 55.8% 44.2% 0.14 0.13-0.15
Meninges (cerebral and spinal) (C70.0-C70.9) 159,403 31,881 38.4% 8.84 8.79-8.88 43,365 8,673 2.2% 97.8% 5.30 5.25-5.35 116,038 23,208 1% 99% 11.97 11.90-12.04
Pituitary (C75.1-C75.2) 74,426 14,885 17.9% 4.46 4.42-4.49 33,614 6,723 0.7% 99.3% 4.07 4.03-4.12 40,812 8,162 0.5% 99.5% 4.93 4.88-4.98
Pineal (C75.3) 1,775 355 0.4% 0.11 0.11-0.12 1,040 208 75% 25% 0.13 0.13-0.14 735 147 44.8% 55.2% 0.09 0.09-0.10
Olfactory tumors of the nasal cavity (C30.0 d) 740 148 0.2% 0.04 0.04-0.05 438 88 100% 0% 0.05 0.05-0.06 302 60 100% 0% 0.03 0.03-0.04
TOTAL 415,411 83,082 100.0% 23.79 23.71-23.86 173,641 34,728 39.5% 60.5% 21.09 20.99-21.20 241,770 48,354 22.7% 77.3% 26.31 26.21-26.42

a. Annual average cases are calculated by dividing the five-year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. The sites referred to in this table are loosely based on the categories and site codes defined in the SEER site/histology validation list.

d. ICD-O-3 histology codes 9522-9523 only.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval

  • Incidence rates were highest for tumors located in the meninges (8.84 per 100,000 population) and lowest for olfactory tumors of the nasal cavity (0.04 per 100,000 population).

  • Incidence rates were higher in females than in males for tumors located in the meninges, pituitary and craniopharyngeal duct, and cranial nerves, while males had higher incidence rates for tumors located in most other locations.

Incidence Rates by Sex and Histology

Incidence rates by sex and histology are presented in Table 3. Incidence rates for all primary brain and other CNS tumors combined were higher among females (26.31 per 100,000 population) than males (21.09 per 100,000 population).

  • The incidence rate of tumors of neuroepithelial tissue was higher in males (7.71 per 100,000 population) than in females (5.55 per 100,000 population).

  • The incidence rate of tumors of meninges was higher in females (12.22 per 100,000 population) than in males (5.56 per 100,000 population).

Average annual age-adjusted incidence rates and incidence rate ratios (male:female) for selected histologies and histology groupings are presented in Supplementary Figure 10 and Fig. 12, respectively.

Fig. 12.

Fig. 12

Incidence Rate Ratios by Sex (Males:Females) for Selected Primary Brain and Other CNS Tumor Histologies, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013–2017

  • Incidence was higher in males for many histologies, such as germ cell tumors (p<0.0001), most glial tumors, lymphomas (p<0.0001), and embryonal tumors (p<0.0001).

  • In addition to non-malignant (p<0.0001) and malignant (p=0.0448) meningiomas, tumors of the pituitary (p<0.0001) were also more common in females than in males.

Distribution and Incidence Rates by CCR, Age, Diagnostic Confirmation, and Behavior

The overall number of reported tumors are listed by CCR in Table 9. While most malignant tumors are diagnosed by histologic confirmation (where the patient receives surgery and diagnosis is confirmed by a pathologist), brain and other CNS tumors may also be diagnosed by radiographic confirmation only (where the tumor was visualized on MRI, CT, X-ray, or other imaging technology but surgery was not performed).

Table 9.

Characteristics of All Brain and Other Central Nervous System Tumors by Central Cancer Registry, Behavior and Diagnostic Confirmation, CBTRUS Statistical Report: U.S. Cancer Statistics – U.S. Cancer Statistics - NPCR and SEER, 2013-2017

State Total Malignant Non-Malignant Average Annual 2013-2017 5-Year Populationa
5-Year Total Annual Average Histologically Confirmedb (%) Radiographically Confirmedc (%) 5-Year Total % Malignant Histologically Confirmed (%) Radiographically Confirmed (%) 5-Year Total % Non-Malignant Histologically Confirmed (%) Radiographically Confirmed (%)
Alabama 4,884 977 62.7% 31.3% 1,806 37.0% 80.0% 7.03% 3,078 63.0% 52.6% 45.5% 4,850,769
Alaska 861 172 47.3% 49.1% 253 29.4% 83.0% 11.07% 608 70.6% 32.4% 65.0% 738,563
Arizona 7,702 1,540 63.3% 32.1% 2,467 32.0% 83.7% 7.54% 5,235 68.0% 53.7% 43.6% 6,809,947
Arkansas 3,617 723 53.4% 42.8% 1,225 33.9% 81.6% 11.27% 2,392 66.1% 38.9% 58.9% 2,977,943
California 45,399 9,080 58.9% 36.5% 13,476 29.7% 85.3% 8.45% 31,923 70.3% 47.8% 48.3% 38,982,847
Colorado 7,754 1,551 49.6% 47.5% 2,010 25.9% 81.3% 13.18% 5,744 74.1% 38.5% 59.5% 5,436,514
Connecticut 3,595 719 68.6% 29.2% 1,244 34.6% 88.9% 8.60% 2,351 65.4% 57.8% 40.1% 3,594,478
Delaware 1,053 211 67.1% 29.7% 384 36.5% 84.1% 10.94% 669 63.5% 57.4% 40.5% 943,733
District of Columbia 762 152 60.5% 36.9% 186 24.4% 90.9% 4.30% 576 75.6% 50.7% 47.4% 672,391
Florida 30,477 6,095 52.5% 43.9% 8,503 27.9% 84.5% 10.31% 21,974 72.1% 40.1% 56.9% 20,278,446
Georgia 13,572 2,714 49.1% 46.4% 3,440 25.4% 83.9% 11.37% 10,132 74.7% 37.3% 58.3% 10,201,631
Hawaii 1,221 244 53.3% 39.2% 305 25.0% 84.3% 9.18% 916 75.0% 43.0% 49.2% 1,421,658
Idaho 2,019 404 60.8% 35.9% 703 34.8% 82.4% 13.51% 1,316 65.2% 49.3% 47.9% 1,657,374
Illinois 17,174 3,435 56.3% 41.4% 4,825 28.1% 87.8% 8.06% 12,349 71.9% 44.0% 54.4% 12,854,528
Indiana 7,820 1,564 53.4% 43.1% 2,555 32.7% 84.4% 10.80% 5,265 67.3% 38.4% 58.8% 6,614,417
Iowa 3,482 696 57.5% 39.9% 1,065 30.6% 84.5% 10.80% 2,417 69.4% 45.6% 52.7% 3,118,098
Kansas 3,570 714 53.1% 44.0% 1,072 30.0% 87.7% 9.79% 2,498 70.0% 38.3% 58.7% 2,903,819
Kentucky 6,929 1,386 48.3% 46.4% 1,968 28.4% 78.9% 12.35% 4,961 71.6% 36.2% 59.9% 4,424,379
Louisiana 6,306 1,261 56.2% 38.6% 1,627 25.8% 85.6% 10.20% 4,679 74.2% 46.0% 48.4% 4,663,459
Maine 1,558 312 66.4% 29.9% 681 43.7% 84.0% 9.84% 877 56.3% 52.8% 45.5% 1,330,161
Maryland 7,303 1,461 61.4% 34.3% 2,160 29.6% 86.5% 6.90% 5,143 70.4% 50.8% 45.8% 5,996,078
Massachusetts 7,586 1,517 69.9% 26.1% 2,888 38.1% 87.4% 7.72% 4,698 61.9% 59.2% 37.4% 6,789,321
Michigan 11,946 2,389 58.1% 36.8% 3,921 32.8% 83.9% 7.96% 8,025 67.2% 45.4% 50.9% 9,925,564
Minnesota 6,379 1,276 70.5% 26.2% 2,310 36.2% 87.4% 8.66% 4,069 63.8% 60.8% 36.2% 5,490,723
Mississippi 3,563 713 57.5% 38.8% 1,060 29.8% 86.1% 10.57% 2,503 70.2% 45.4% 50.8% 2,986,222
Missouri 8,130 1,626 54.1% 42.0% 2,500 30.8% 85.7% 8.88% 5,630 69.2% 40.1% 56.7% 6,075,301
Montana 1,471 294 54.7% 41.5% 485 33.0% 82.1% 13.40% 986 67.0% 41.3% 55.3% 1,029,864
Nebraska 2,186 437 58.3% 38.4% 789 36.1% 84.2% 9.51% 1,397 63.9% 43.7% 54.7% 1,893,915
Nevada 3,052 610 57.1% 37.7% 1,075 35.2% 83.1% 8.37% 1,977 64.8% 43.0% 53.7% 2,887,725
New Hampshire 1,752 350 62.9% 34.9% 631 36.0% 91.4% 4.75% 1,121 64.0% 46.8% 51.8% 1,331,850
New Jersey 12,870 2,574 55.1% 40.2% 3,708 28.8% 86.7% 8.79% 9,162 71.2% 42.3% 52.9% 8,960,161
New Mexico 1,679 336 65.0% 28.6% 533 31.8% 86.7% 6.94% 1,146 68.2% 55.0% 38.7% 2,084,827
New York 30,630 6,126 51.6% 45.1% 7,961 26.0% 84.1% 11.15% 22,669 74.0% 40.1% 57.0% 19,798,225
North Carolina 13,440 2,688 54.6% 42.1% 3,819 28.4% 85.3% 9.48% 9,621 71.6% 42.4% 55.1% 10,052,570
North Dakota 812 162 49.5% 47.7% 271 33.4% 82.3% 13.28% 541 66.6% 33.1% 64.9% 745,473
Ohio 13,664 2,733 64.6% 30.9% 4,987 36.5% 85.8% 7.28% 8,677 63.5% 52.4% 44.5% 11,609,755
Oklahoma 4,520 904 55.1% 41.0% 1,494 33.0% 82.5% 9.84% 3,026 67.0% 41.5% 56.4% 3,896,254
Oregon 4,721 944 67.1% 28.6% 1,776 37.6% 83.6% 7.21% 2,945 62.4% 57.1% 41.4% 4,025,128
Pennsylvania 19,748 3,950 50.0% 45.6% 5,829 29.5% 81.5% 10.14% 13,919 70.5% 36.8% 60.5% 12,790,507
Rhode Island 1,172 234 65.5% 30.8% 443 37.8% 87.6% 7.90% 729 62.2% 52.1% 44.7% 1,056,137
South Carolina 6,523 1,305 52.1% 42.9% 1,933 29.6% 83.7% 9.88% 4,590 70.4% 38.7% 56.8% 4,893,447
South Dakota 1,036 207 47.6% 49.2% 332 32.0% 79.2% 15.96% 704 68.0% 32.7% 64.9% 855,442
Tennessee 9,059 1,812 51.8% 45.5% 2,532 27.9% 86.0% 9.72% 6,527 72.0% 38.6% 59.4% 6,597,383
Texas 33,471 6,694 50.1% 43.8% 9,426 28.2% 80.3% 13.24% 24,045 71.8% 38.3% 55.7% 27,419,611
Utah 5,059 1,012 45.4% 53.4% 1,011 20.0% 84.7% 13.25% 4,048 80.0% 35.6% 63.4% 2,993,941
Vermont 913 183 57.9% 39.2% 278 30.4% 89.9% 4.68% 635 69.5% 43.9% 54.3% 624,635
Virginia 8,844 1,769 63.3% 32.8% 3,019 34.1% 85.8% 6.79% 5,825 65.9% 51.6% 46.3% 8,365,952
Washington 12,240 2,448 45.6% 50.1% 3,041 24.8% 81.7% 11.28% 9,199 75.2% 33.7% 63.0% 7,169,967
West Virginia 2,538 508 54.5% 41.9% 824 32.5% 87.0% 9.95% 1,714 67.5% 38.9% 57.2% 1,836,844
Wisconsin 8,675 1,735 49.8% 46.7% 2,427 28.0% 83.4% 11.66% 6,248 72.0% 36.7% 60.4% 5,763,218
Wyoming 674 135 65.1% 33.4% 226 33.5% 87.6% 9.73% 448 66.5% 53.8% 45.3% 583,200
Total 415,411 83,082 55.3% 40.6% 123,484 29.7% 84.3% 9.72% 291,927 70.3% 43.0% 53.7% 321,004,396

a. Population estimates were obtained from the United States Bureau of the Census available on the SEER program website.

b. Histologic confirmation includes tumors classified as having diagnosis confirmed by: positive histology, positive cytology, positive histology plus – positive immunophenotyping and/or positive genetic studies, or positive microscopic confirmation, method not specified.

c. Radiographic confirmation includes tumors classified as having diagnosis confirmed by Radiography and/or other imaging techniques without microscopic confirmation.

- Counts and rates are not presented when fewer than 16 cases were reported for the specific category, or where the inclusion of the count and rate would allow for back-calculation of suppressed values. The suppressed cases are included in the counts and rates for Totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program.

  • Approximately 70.3% of tumors were non-malignant, but there was variation by cancer registry (range: 56.3%-80.0%).

  • Overall, 55.3% of tumors were histologically confirmed. A larger proportion of malignant tumors were histologically confirmed (84.3%) compared to non-malignant tumors (43.0%).

  • A slight majority of non-malignant brain and other CNS tumors were radiographically confirmed (53.7%).

The overall average annual age-adjusted incidence rates by age, behavior, and CCR are presented in Tables 9 and 10 and Fig. 13. Incidence rates for all primary brain and other CNS tumors combined are presented in Supplementary Figure 11.

Table 10.

Average Annual Age-Adjusted Incidence Ratesa with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Age at Diagnosis, Behavior, and Central Cancer Registry, CBTRUS Statistical Report: U.S. Cancer Statistics – U.S. Cancer Statistics - NPCR and SEER, 2013-2017

State 0-19 Years 20+ Years All Ages
Malignant Non-Malignant All Tumors Malignant Non-Malignant All Tumors Malignant Non-Malignant All Tumors
Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI Rate 95% CI
Alabama 3.72 3.26-4.24 1.45 1.17-1.78 5.17 4.62-5.77 7.81 7.41-8.21 15.22 14.66-15.79 23.02 22.34-23.72 6.63 6.32-6.96 11.27 10.86-11.69 17.9 17.39-18.43
Alaska 3.2 2.20-4.50 3.55 2.47-4.93 6.76 5.24-8.56 8.43 7.29-9.70 23.32 21.33-25.44 31.75 29.44-34.19 6.93 6.06-7.89 17.65 16.19-19.20 24.58 22.87-26.38
Arizona 2.94 2.59-3.31 2.53 2.21-2.88 5.47 5.00-5.97 7.83 7.50-8.17 18.33 17.81-18.86 26.15 25.54-26.78 6.42 6.17-6.69 13.8 13.42-14.18 20.22 19.76-20.69
Arkansas 3.82 3.23-4.48 2.55 2.07-3.10 6.37 5.60-7.21 8.74 8.22-9.30 19.18 18.38-20.01 27.93 26.97-28.91 7.33 6.92-7.77 14.41 13.82-15.02 21.74 21.02-22.48
California 3.09 2.94-3.25 2.26 2.14-2.40 5.35 5.15-5.56 8.08 7.93-8.23 21.12 20.88-21.36 29.19 28.91-29.48 6.65 6.53-6.76 15.71 15.54-15.89 22.36 22.15-22.57
Colorado 3.42 3.00-3.88 2.47 2.11-2.87 5.89 5.33-6.49 8.54 8.14-8.96 27.48 26.75-28.23 36.02 35.19-36.88 7.07 6.76-7.40 20.31 19.77-20.85 27.38 26.76-28.01
Connecticut 3.79 3.17-4.50 2.62 2.11-3.20 6.41 5.60-7.31 9.15 8.60-9.72 19.24 18.42-20.08 28.39 27.41-29.40 7.61 7.18-8.06 14.47 13.87-15.09 22.08 21.34-22.84
Delaware 4.04 2.96-5.39 2.69 1.83-3.82 6.73 5.31-8.42 8.31 7.42-9.28 16.28 15.00-17.65 24.6 23.02-26.25 7.09 6.37-7.86 12.39 11.43-13.40 19.47 18.27-20.73
District of Columbia 3.98 2.63-5.78 2.84 1.69-4.45 6.82 4.98-9.10 6.46 5.47-7.58 23.53 21.56-25.62 29.99 27.77-32.33 5.75 4.93-6.67 17.59 16.15-19.14 23.34 21.67-25.11
Florida 3.72 3.47-3.98 3.04 2.82-3.27 6.76 6.43-7.11 8.3 8.11-8.49 23.45 23.12-23.78 31.75 31.37-32.13 6.99 6.83-7.14 17.59 17.35-17.84 24.58 24.29-24.87
Georgia 3.52 3.22-3.85 2.88 2.60-3.17 6.4 5.99-6.84 7.79 7.51-8.08 26.1 25.57-26.64 33.89 33.29-34.50 6.57 6.34-6.79 19.44 19.05-19.83 26.01 25.56-26.45
Hawaii 1.64 1.02-2.48 1.83 1.17-2.72 3.47 2.54-4.62 5.94 5.25-6.70 18.88 17.62-20.21 24.82 23.37-26.34 4.71 4.18-5.29 13.99 13.06-14.96 18.7 17.62-19.82
Idaho 3.62 2.90-4.46 2.17 1.62-2.85 5.79 4.87-6.84 9.62 8.85-10.43 20.12 19.00-21.29 29.73 28.37-31.15 7.89 7.31-8.52 14.97 14.15-15.82 22.87 21.86-23.91
Illinois 3.3 3.03-3.59 2.59 2.35-2.85 5.89 5.53-6.28 8.38 8.12-8.64 23.82 23.38-24.26 32.19 31.69-32.70 6.92 6.72-7.12 17.73 17.41-18.05 24.65 24.27-25.03
Indiana 3.15 2.79-3.54 2.32 2.02-2.66 5.47 5.00-5.98 8.73 8.37-9.11 19.59 19.04-20.15 28.32 27.66-28.99 7.13 6.85-7.42 14.63 14.23-15.04 21.76 21.27-22.26
Iowa 3.6 2.98-4.31 3.53 2.92-4.23 7.13 6.25-8.11 9.13 8.54-9.75 22.85 21.90-23.84 31.99 30.86-33.15 7.55 7.09-8.03 17.31 16.60-18.04 24.86 24.01-25.72
Kansas 3.64 3.07-4.28 2.57 2.09-3.11 6.21 5.46-7.03 8.12 7.59-8.68 21.39 20.52-22.29 29.52 28.49-30.57 6.84 6.42-7.27 15.99 15.35-16.65 22.83 22.07-23.61
Kentucky 4.23 3.71-4.80 3.53 3.05-4.05 7.76 7.05-8.52 9.63 9.17-10.11 27.07 26.28-27.87 36.7 35.79-37.63 8.08 7.72-8.46 20.31 19.74-20.90 28.4 27.71-29.09
Louisiana 3.52 3.07-4.02 2.79 2.39-3.24 6.31 5.70-6.97 7.75 7.34-8.18 25.22 24.47-25.99 32.97 32.11-33.84 6.54 6.22-6.87 18.79 18.24-19.35 25.32 24.69-25.97
Maine 4.3 3.29-5.51 1.2 0.71-1.90 5.49 4.35-6.84 10.01 9.20-10.88 14.44 13.43-15.50 24.45 23.15-25.81 8.37 7.72-9.07 10.64 9.91-11.42 19.01 18.02-20.04
Maryland 3.16 2.77-3.59 2.13 1.82-2.49 5.3 4.79-5.84 8.08 7.71-8.46 20.96 20.37-21.57 29.04 28.34-29.75 6.67 6.38-6.96 15.56 15.13-16.00 22.23 21.71-22.76
Massachusetts 3.54 3.13-3.98 2.19 1.88-2.53 5.73 5.21-6.28 9.25 8.88-9.62 16.17 15.69-16.66 25.42 24.81-26.03 7.61 7.32-7.90 12.16 11.80-12.52 19.77 19.31-20.23
Michigan 3.31 2.99-3.64 1.9 1.66-2.15 5.2 4.81-5.62 8.47 8.18-8.76 19.12 18.68-19.56 27.59 27.06-28.12 6.99 6.76-7.22 14.18 13.86-14.50 21.16 20.77-21.56
Minnesota 3.89 3.45-4.38 2.44 2.09-2.83 6.34 5.76-6.95 9.3 8.88-9.72 17.82 17.25-18.40 27.11 26.41-27.83 7.75 7.42-8.08 13.41 12.99-13.84 21.15 20.62-21.69
Mississippi 2.55 2.08-3.09 2.22 1.79-2.73 4.77 4.12-5.49 8.13 7.61-8.68 20.83 19.98-21.70 28.96 27.96-29.98 6.53 6.13-6.94 15.49 14.87-16.12 22.02 21.28-22.77
Missouri 4.15 3.71-4.63 2.22 1.90-2.57 6.37 5.82-6.96 8.64 8.27-9.02 22.25 21.64-22.86 30.89 30.18-31.61 7.35 7.06-7.66 16.5 16.06-16.95 23.86 23.32-24.40
Montana 2.69 1.86-3.76 2.2 1.46-3.18 4.89 3.75-6.27 9.99 9.05-11.01 22.23 20.77-23.76 32.22 30.47-34.03 7.89 7.17-8.67 16.48 15.42-17.60 24.38 23.09-25.72
Nebraska 4 3.27-4.84 2.96 2.34-3.70 6.96 5.98-8.04 9.13 8.44-9.86 18.35 17.35-19.40 27.48 26.26-28.74 7.66 7.12-8.22 13.94 13.19-14.71 21.59 20.67-22.54
Nevada 3.48 2.90-4.14 1.88 1.46-2.39 5.36 4.64-6.17 8.16 7.63-8.71 17 16.23-17.80 25.16 24.22-26.12 6.81 6.40-7.25 12.66 12.10-13.25 19.48 18.78-20.20
New Hampshire 4.62 3.59-5.84 2.06 1.42-2.91 6.68 5.44-8.11 9.46 8.66-10.31 18.94 17.78-20.15 28.39 26.98-29.86 8.07 7.42-8.76 14.1 13.25-14.99 22.16 21.09-23.28
New Jersey 3.75 3.40-4.13 3.1 2.78-3.45 6.85 6.37-7.36 9.04 8.73-9.36 24.55 24.03-25.09 33.6 32.99-34.22 7.53 7.28-7.78 18.4 18.02-18.79 25.92 25.47-26.39
New Mexico 2.43 1.82-3.17 1.84 1.32-2.50 4.27 3.45-5.22 7.05 6.41-7.73 16.74 15.74-17.79 23.79 22.59-25.03 5.72 5.23-6.25 12.47 11.73-13.23 18.19 17.30-19.11
New York 4.05 3.80-4.32 4.08 3.83-4.35 8.14 7.78-8.51 8.67 8.47-8.88 27.39 27.01-27.76 36.06 35.63-36.49 7.35 7.18-7.52 20.7 20.43-20.98 28.05 27.73-28.37
North Carolina 3.57 3.25-3.91 2.46 2.20-2.75 6.03 5.61-6.47 8.26 7.98-8.55 23.28 22.80-23.77 31.54 30.98-32.11 6.92 6.69-7.14 17.31 16.96-17.67 24.22 23.81-24.65
North Dakota 3.27 2.25-4.61 2.92 1.94-4.22 6.19 4.73-7.96 8.21 7.17-9.36 18.84 17.18-20.61 27.05 25.08-29.13 6.79 5.99-7.68 14.27 13.05-15.57 21.06 19.59-22.62
Ohio 4.18 3.86-4.53 2.67 2.41-2.95 6.85 6.43-7.29 9 8.72-9.28 17.49 17.10-17.88 26.49 26.01-26.97 7.61 7.40-7.84 13.24 12.95-13.53 20.85 20.49-21.22
Oklahoma 3.45 2.97-3.99 2.1 1.73-2.53 5.55 4.94-6.22 8.45 7.99-8.93 19.43 18.71-20.17 27.88 27.03-28.76 7.02 6.66-7.39 14.46 13.94-15.00 21.48 20.84-22.13
Oregon 4.03 3.48-4.64 2.72 2.27-3.22 6.75 6.04-7.52 9.3 8.83-9.78 17 16.35-17.66 26.29 25.50-27.11 7.79 7.42-8.17 12.9 12.42-13.39 20.69 20.08-21.31
Pennsylvania 4.18 3.86-4.52 2.32 2.09-2.57 6.5 6.10-6.92 9.33 9.07-9.60 24.94 24.50-25.38 34.27 33.76-34.78 7.85 7.64-8.07 18.45 18.13-18.77 26.3 25.92-26.69
Rhode Island 2.68 1.83-3.79 1.86 1.18-2.78 4.54 3.42-5.90 9.03 8.15-9.98 15.93 14.73-17.19 24.95 23.46-26.51 7.21 6.53-7.94 11.89 11.01-12.82 19.09 17.98-20.26
South Carolina 3.31 2.87-3.80 2.45 2.08-2.87 5.76 5.17-6.39 8.37 7.97-8.79 22.13 21.46-22.81 30.5 29.72-31.30 6.92 6.61-7.25 16.49 16.00-16.99 23.41 22.82-24.00
South Dakota 3.37 2.41-4.59 1.71 1.04-2.64 5.08 3.88-6.54 8.36 7.40-9.42 20.37 18.82-22.02 28.73 26.89-30.67 6.93 6.18-7.74 15.02 13.89-16.22 21.95 20.58-23.38
Tennessee 3.5 3.11-3.92 2.63 2.29-3.00 6.13 5.61-6.68 8.29 7.94-8.65 23.97 23.37-24.59 32.26 31.57-32.97 6.92 6.64-7.20 17.85 17.41-18.30 24.77 24.25-25.30
Texas 3.62 3.44-3.81 2.73 2.57-2.90 6.35 6.11-6.60 8.25 8.07-8.43 24.21 23.90-24.53 32.46 32.09-32.83 6.92 6.78-7.06 18.05 17.82-18.28 24.97 24.70-25.24
Utah 3.02 2.55-3.54 3.05 2.58-3.59 6.07 5.40-6.80 9.15 8.54-9.80 42.22 40.88-43.59 51.37 49.89-52.88 7.39 6.93-7.87 30.98 30.02-31.97 38.37 37.30-39.47
Vermont 2.92 1.76-4.55 3.46 2.25-5.11 6.38 4.64-8.56 9.45 8.28-10.74 22.66 20.80-24.64 32.11 29.90-34.45 7.58 6.67-8.58 17.15 15.78-18.63 24.73 23.07-26.49
Virginia 3.36 3.02-3.73 1.83 1.58-2.11 5.19 4.76-5.65 7.98 7.67-8.29 17.09 16.63-17.55 25.07 24.52-25.62 6.65 6.41-6.90 12.71 12.38-13.05 19.36 18.95-19.78
Washington 4.35 3.92-4.80 3.37 3.00-3.77 7.71 7.15-8.31 9.3 8.94-9.67 31.85 31.17-32.53 41.15 40.38-41.92 7.88 7.59-8.17 23.68 23.18-24.18 31.56 30.99-32.13
West Virginia 4.1 3.28-5.06 2.39 1.78-3.14 6.48 5.44-7.66 9.05 8.38-9.76 20.86 19.83-21.94 29.91 28.67-31.19 7.63 7.09-8.20 15.56 14.80-16.35 23.19 22.26-24.16
Wisconsin 2.8 2.43-3.22 2.07 1.76-2.43 4.88 4.38-5.41 9.28 8.89-9.69 26.03 25.36-26.71 35.31 34.53-36.10 7.42 7.12-7.74 19.16 18.67-19.65 26.58 26.01-27.17
Wyoming -- -- -- -- 3.14 2.01-4.67 9.18 7.95-10.56 19.62 17.76-21.61 28.8 26.55-31.19 7.1 6.18-8.13 14.34 12.99-15.78 21.44 19.80-23.18
TOTAL 3.55 3.49-3.61 2.6 2.55-2.65 6.14 6.07-6.22 8.5 8.45-8.55 22.38 22.30-22.47 30.89 30.79-30.99 7.08 7.04-7.12 16.71 16.64-16.77 23.79 23.71-23.86

a. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

- Counts are not presented when fewer than 16 cases were reported for the specific category, or where the inclusion of the count and rate would allow for back-calculation of suppressed values. The suppressed cases are included in the counts and rates for Totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval

Fig. 13.

Fig. 13

Average Annual Age-Adjusted Incidence Ratesa of A) Malignant and B) Non-Malignant Primary Brain and Other CNS Tumors by Central Cancer Registry, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • There was less variation by region for malignant tumor incidence rates (Fig. 13A) compared to incidence rates for non-malignant tumors (Fig. 13B). CCR and regional variations likely reflect differences in reporting and case ascertainment practices, including state-level adoption of computer-aided registration and data linkages.

  • The overall AAAIR of all tumors (malignant and non-malignant) for each individual CCR ranged from 17.9 to 38.4 per 100,000 population.

  • AAAIR of all primary malignant tumors ranged from 4.71 to 8.37 per 100,000 population, and AAAIR of all primary non-malignant tumors ranged from 10.64 to 30.98 per 100,000 population.

  • Among adults 20 years of age and older, CCR-specific incidence rates ranged from 5.94 to 10.01 per 100,000 population for malignant tumors and from 14.44 to 42.22 per 100,000 population for non-malignant tumors.

  • In persons less than 20 years of age, incidence rates ranged from 1.64 to 4.62 per 100,000 population for malignant tumors and from 1.2 to 4.08 per 100,000 population for non-malignant tumors.

Distribution by Histology, WHO Grade Completeness, Diagnostic Confirmation, and Treatment Completeness

The distribution of reported tumors with histologically confirmed diagnosis from 2013 to 2017 is presented by histology and reported WHO grade in Table 11.

Table 11.

Distribution of Histologically-Confirmed Brain and Other Central Nervous System Tumors by WHO Grade Completeness, Treatment Information Completeness, and Major Histology Grouping, CBTRUS Statistical Report: U.S. Cancer Statistics – U.S. Cancer Statistics - NPCR and SEER, 2013-2017

Histology Number of Newly Diagnosed Tumors (2013-2017) Histologically Confirmeda (%) WHO Grade Completeness (%) Assigned WHO Grade Radiation Information Completenessc (%) Surgical Extent of Resection Information Complete Completenessd (%)
Completeb Incomplete Not Applicable I II III IV
Tumors of Neuroepithelial Tissue 113,056 89.2% 87.0% 11.5% 1.5% 10.6% 13.7% 13.0% 62.8% 55.4% 97.8%
Pilocytic Astrocytoma 5,167 90.1% 87.9% 11.7% 0.4% 93.9% 5.1% 0.8% 0.3% 5.8% 99.0%
Diffuse Astrocytoma 7,428 92.8% 86.0% 13.8% 0.1% 3.3% 67.5% 17.9% 11.2% 50.1% 97.4%
Anaplastic Astrocytoma 7,116 99.3% 95.6% 4.4% 0.1% 0.1% 1.2% 90.1% 8.6% 77.6% 99.1%
Unique Astrocytoma Variants 1,123 75.4% 77.7% 22.1% 0.2% 21.0% 54.0% 20.1% 5.0% 21.0% 97.8%
Malignant 769 87.3% 79.7% 20.0% 0.3% 2.8% 66.4% 24.7% 6.2% 30.1% 97.5%
Non-Malignant 354 49.7% 69.9% 30.1% 0.0% 100.0% 0.0% 0.0% 0.0% 1.8% 98.3%
Glioblastoma 60,056 93.4% 90.5% 9.5% 0.0% 0.2% 0.2% 0.7% 99.0% 69.6% 97.5%
Oligodendroglioma 3,698 96.8% 93.7% 6.3% 0.0% 1.6% 88.3% 6.3% 3.9% 36.9% 98.0%
Anaplastic Oligodendroglioma 1,859 99.1% 95.7% 4.3% 0.0% 0.0% 3.2% 88.8% 8.0% 71.8% 98.8%
Oligoastrocytic Tumors 1,572 99.0% 94.8% 5.2% 0.0% 0.7% 48.3% 42.7% 8.2% 59.7% 99.5%
Ependymal Tumors 6,843 88.4% 87.5% 12.4% 0.1% 36.0% 48.6% 14.5% 0.9% 26.1% 98.5%
Malignant 3,972 93.6% 90.2% 9.7% 0.1% 2.5% 73.5% 22.7% 1.3% 38.5% 98.7%
Non-Malignant 2,871 81.2% 83.1% 16.8% 0.0% 93.9% 5.6% 0.3% 0.3% 8.7% 98.2%
Glioma Malignant, NOS 8,093 36.3% 50.9% 48.0% 1.1% 15.0% 26.7% 22.4% 35.9% 28.2% 97.4%
Choroid Plexus Tumors 827 88.0% 77.2% 22.8% 0.0% 64.2% 19.3% 15.9% 0.7% 4.7% 98.1%
Malignant 128 97.7% 80.8% 19.2% 0.0% 5.0% 3.0% 88.1% 4.0% 15.0% 100.0%
Non-Malignant 699 86.3% 76.4% 23.6% 0.0% 77.2% 22.8% 0.0% 0.0% 2.8% 97.8%
Other Neuroepithelial Tumors 107 94.4% 57.4% 41.6% 1.0% 8.6% 55.2% 24.1% 12.1% 39.4% 97.5%
Malignant 66 98.5% 47.7% 50.8% 1.5% 9.7% 22.6% 45.2% 22.6% 54.1% 96.0%
Non-Malignant 41 87.8% 75.0% 25.0% 0.0% 7.4% 92.6% 0.0% 0.0% 15.8% 100.0%
Neuronal and Mixed Neuronal Glial Tumors 4,934 92.2% 65.4% 18.1% 16.5% 81.0% 15.1% 3.2% 0.6% 15.5% 98.4%
Malignant 947 98.5% 16.5% 4.8% 78.7% 28.6% 9.1% 55.2% 7.1% 61.7% 97.4%
Non-Malignant 3,987 90.7% 78.0% 21.5% 0.5% 83.9% 15.5% 0.4% 0.3% 4.7% 98.6%
Tumors of the Pineal Region 787 77.5% 0.0% 0.0% 100.0% -- -- -- -- 40.9% 98.3%
Malignant 447 96.9% 0.0% 0.0% 100.0% -- -- -- -- 66.3% 98.1%
Non-Malignant 340 52.1% 0.0% 0.0% 100.0% -- -- -- -- 8.6% 98.4%
Embryonal Tumors 3,446 98.1% 77.5% 21.7% 0.7% 1.3% 0.2% 1.5% 97.1% 62.8% 97.6%
Tumors of Cranial and Spinal Nerves 35,600 49.7% 36.4% 63.6% 0.0% 99.2% 0.5% 0.2% 0.2% 17.4% 98.3%
Nerve Sheath Tumors 35,560 49.7% 36.4% 63.6% 0.0% 99.2% 0.5% 0.2% 0.2% 17.4% 98.3%
Malignant 223 78.9% 22.7% 77.3% 0.0% 57.5% 15.0% 15.0% 12.5% 34.7% 85.7%
Non-Malignant 35,337 49.6% 36.5% 63.5% 0.0% 99.4% 0.4% 0.1% 0.1% 17.3% 98.3%
Other Tumors of Cranial and Spinal Nerves 40 42.5% 23.5% 76.5% 0.0% 100.0% 0.0% 0.0% 0.0% 2.5% 100.0%
Tumors of Meninges 163,619 40.1% 79.9% 20.1% 0.1% 80.1% 17.7% 2.1% 0.2% 7.2% 97.7%
Meningioma 159,038 38.8% 81.3% 18.7% 0.0% 80.3% 17.9% 1.6% 0.1% 6.9% 97.6%
Malignant 1,750 79.2% 85.3% 14.6% 0.1% 21.2% 17.2% 60.6% 1.1% 38.8% 84.9%
Non-Malignant 157,288 38.3% 81.2% 18.8% 0.0% 81.7% 18.0% 0.2% 0.1% 6.6% 97.7%
Mesenchymal Tumors 1,462 74.7% 55.7% 43.8% 0.5% 9.4% 43.3% 42.9% 4.4% 32.4% 98.1%
Primary Melanocytic Lesions 108 88.9% 12.5% 82.3% 5.2% 58.3% 33.3% 0.0% 8.3% 45.4% 94.7%
Other Neoplasms Related to the Meninges 3,011 91.9% 59.1% 39.9% 1.0% 99.3% 0.4% 0.1% 0.2% 6.9% 98.4%
Lymphoma and Hematopoietic Neoplasms 8,150 94.9% 7.7% 90.7% 1.6% 100.0% 0.0% 0.0% 0.0% 20.7% 99.1%
Lymphoma 7,919 95.0% 8.0% 91.3% 0.7% 100.0% 0.0% 0.0% 0.0% 20.3% 99.1%
Other Hematopoietic Neoplasms 231 88.7% 0.9% 79.6% 19.5% 100.0% 0.0% 0.0% 0.0% 33.5% 99.2%
Germ Cell Tumors and Cysts 1,585 81.1% 3.8% 53.7% 42.5% 18.4% 6.1% 6.1% 69.4% 47.9% 98.2%
Germ Cell Tumors, Cysts and Heterotopias 1,585 81.1% 3.8% 53.7% 42.5% 18.4% 6.1% 6.1% 69.4% 47.9% 98.2%
Malignant 1,095 88.0% 4.4% 42.5% 53.1% 4.8% 7.1% 7.1% 81.0% 67.9% 98.9%
Non-Malignant 490 65.7% 2.2% 87.0% 10.9% 100.0% 0.0% 0.0% 0.0% 2.8% 96.6%
Tumors of Sellar Region 73,340 46.9% 0.5% 0.5% 99.0% 100.0% 0.0% 0.0% 0.0% 3.0% 97.7%
Tumors of the Pituitary 70,211 45.2% 0.0% 0.0% 100.0% -- -- -- -- 2.2% 97.7%
Malignant 142 66.9% 0.0% 0.0% 100.0% -- -- -- -- 19.2% 84.8%
Non-Malignant 70,069 45.2% 0.0% 0.0% 100.0% -- -- -- -- 2.2% 97.7%
Craniopharyngioma 3,129 84.0% 6.4% 6.5% 87.1% 100.0% 0.0% 0.0% 0.0% 22.0% 97.9%
Unclassified Tumors 20,061 17.4% 4.7% 87.0% 8.3% 63.6% 9.1% 9.7% 17.6% 3.9% 69.8%
Hemangioma 5,731 29.6% 2.5% 97.1% 0.4% 88.4% 7.0% 4.7% 0.0% 2.2% 97.6%
Neoplasm Unspecified 14,136 12.2% 6.4% 78.0% 15.6% 56.4% 10.9% 10.9% 21.8% 4.8% 59.9%
Malignant 6,587 8.2% 8.5% 85.8% 5.7% 17.4% 13.0% 23.9% 45.7% 9.0% 42.5%
Non-Malignant 7,549 15.6% 5.4% 74.5% 20.1% 84.4% 9.4% 1.6% 4.7% 2.8% 75.7%
All Other 194 37.6% 16.4% 63.0% 20.5% 41.7% 0.0% 16.7% 41.7% 10.6% 92.1%
TOTAL 415,411 55.6% 65.2% 18.7% 16.1% 39.5% 14.5% 8.5% 37.5% 21.0% 95.9%
Malignant 123,484 85.5% 83.9% 14.1% 2.0% 6.0% 13.8% 14.6% 65.5% 55.1% 92.1%
Non-Malignant 291,927 43.0% 50.2% 22.4% 27.4% 84.1% 15.4% 0.3% 0.1% 6.8% 97.1%

a. Histologic confirmation includes tumors classified as diagnosis confirmed by: positive histology, positive cytology, positive histology plus – positive immunophenotyping and/or positive genetic studies, or positive microscopic confirmation, method not specified.

b. Completeness is defined as having an assigned code that corresponds with a WHO grade as defined by the American Joint Commission on Cancer’s Collaborative Staging schema.

c. Radiation is defined using a recoded variable based on NAACCR Item #1360 (http://datadictionary.naaccr.org/default.aspx?c=10#136). Completeness is defined as having a value other than ‘none’ or ‘unknown.’

d. Surgery is defined using a recoded variable based on NAACCR Item #1290 (http://datadictionary.naaccr.org/default.aspx?c=10#1290). Please see the SEER site-specific surgery codes for more information on coding for this variable (https://seer.cancer.gov/archive/tools/SEER2003.surg.prim.site.codes.pdf). Completeness is defined as having a value other than ‘unknown.’

- Percentages are not presented when category is not applicable.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; CNS, central nervous system; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; WHO, World Health Organization

  • 65.2% of tumors had complete WHO grade information, but there was substantial variation by histology.

  • The histologic types with the highest WHO grade completeness were anaplastic oligodendroglioma (93.7%), anaplastic astrocytoma (95.7%), and oligoastrocytic Tumors (94.8%).

Incidence by Urban or Rural Residence at Time of Diagnosis

Incidence counts and average annual age-adjusted rates for brain and other CNS tumors are presented by urban/rural residence and histology in Supplementary Table 9. Incidence of selected histologies by urban/rural residence is presented in Figure 14.

Fig. 14.

Fig. 14

Average Annual Age-Adjusted Incidence Ratesa and Incidence Rate Ratios with 95% Confidence Intervals of Selected Primary Brain and Other CNS Tumor Histologies by Urban Or Rural Residence at Time of Diagnosisb, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • Overall incidence of brain and other CNS tumors was 11.7% higher in urban areas as compared to rural areas (23.78 per 100,000 and 21.29 per 100,000, respectively, p<0.0001).

  • Incidence of malignant brain and other CNS tumors was slightly higher in urban areas (6.95 per 100,000) as compared to rural areas (6.89 per 100,000, p=0.2936).

  • Incidence of non-malignant brain and other CNS tumors was 17% higher in urban areas as compared to rural areas (16.84 per 100,000 and 14.4 per 100,000, respectively, p<0.0001).

  • Incidence of glioblastoma (2.1%, p=0.0715) was higher in urban as compared to rural areas.

  • Predominantly non-malignant histologies were primarily diagnosed more frequently in urban areas, including meningioma (8.91% higher, p<0.0001), nerve sheath tumors (2.05% higher, p<0.0001), and tumors of the pituitary (4.25% higher, p<0.0001).

Distribution of Tumors in Puerto Rico

The distribution of brain and other CNS tumors diagnosed among residents of Puerto Rico by histology is presented in Supplementary Figure 12.

  • Approximately 38.3% of tumors were malignant, and 61.7% were non-malignant.

  • Non-malignant meningioma was the most common tumor type (26%), followed by glioblastoma (17.9%).

Incidence Rates by Race and Histology

Incidence rates by race and histology are presented in Table 12.

Table 12.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and Racec, CBTRUS Statistical Report: U.S. Cancer Statistics – U.S. Cancer Statistics - NPCR and SEER, 2013-2017

Histology White Black American Indian/Alaska Native Asian/Pacific Islander
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Tumors of Neuroepithelial Tissue 99,024 19,805 7.10 7.06-7.15 8,501 1,700 3.92 3.84-4.01 632 126 3.10 2.85-3.37 2,561 512 2.69 2.59-2.80
Pilocytic Astrocytoma 4,195 839 0.38 0.37-0.39 621 124 0.26 0.24-0.29 35 7 0.14 0.10-0.19 120 24 0.13 0.11-0.16
Diffuse Astrocytoma 6,406 1,281 0.49 0.48-0.50 592 118 0.27 0.25-0.29 59 12 0.28 0.21-0.36 191 38 0.20 0.17-0.23
Anaplastic Astrocytoma 6,300 1,260 0.47 0.45-0.48 466 93 0.22 0.20-0.24 38 8 0.18 0.12-0.25 189 38 0.19 0.16-0.22
Unique Astrocytoma Variants 896 179 0.07 0.07-0.08 142 28 0.06 0.05-0.07 -- -- -- -- 47 9 0.05 0.04-0.07
Malignant 639 128 0.05 0.05-0.06 76 15 0.03 0.03-0.04 -- -- -- -- 31 6 0.03 0.02-0.04
Non-Malignant 257 51 0.02 0.02-0.03 66 13 0.03 0.02-0.04 -- -- -- -- 16 3 0.02 0.01-0.03
Glioblastoma 54,211 10,842 3.51 3.48-3.54 3,683 737 1.77 1.71-1.83 264 53 1.49 1.30-1.69 1,107 221 1.18 1.11-1.25
Oligodendroglioma 3,242 648 0.26 0.25-0.27 244 49 0.12 0.10-0.13 29 6 0.13 0.09-0.19 85 17 0.08 0.07-0.10
Anaplastic Oligodendroglioma 1,627 325 0.12 0.12-0.13 102 20 0.05 0.04-0.06 -- -- -- -- 67 13 0.07 0.05-0.09
Oligoastrocytic Tumors 1,398 280 0.11 0.11-0.12 85 17 0.04 0.03-0.05 -- -- -- -- 43 9 0.04 0.03-0.06
Ependymal Tumors 5,837 1,167 0.46 0.45-0.47 576 115 0.26 0.24-0.28 41 8 0.18 0.13-0.25 179 36 0.18 0.16-0.21
Malignant 3,316 663 0.26 0.25-0.27 381 76 0.17 0.15-0.19 18 4 0.08 0.05-0.12 129 26 0.13 0.11-0.16
Non-Malignant 2,521 504 0.19 0.19-0.20 195 39 0.09 0.08-0.10 23 5 0.11 0.07-0.16 50 10 0.05 0.04-0.07
Glioma Malignant, NOS 6,703 1,341 0.53 0.52-0.54 881 176 0.40 0.37-0.43 55 11 0.26 0.19-0.34 205 41 0.22 0.19-0.25
Choroid Plexus Tumors 697 139 0.06 0.05-0.06 72 14 0.03 0.02-0.04 -- -- -- -- -- -- -- --
Malignant 99 20 0.01 0.01-0.01 18 4 0.01 0.00-0.01 -- -- -- -- -- -- -- --
Non-Malignant 598 120 0.05 0.05-0.05 54 11 0.02 0.02-0.03 -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 81 16 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Malignant 48 10 0.00 0.00-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 33 7 0.00 0.00-0.00 -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 4,064 813 0.34 0.33-0.35 514 103 0.23 0.21-0.25 35 7 0.14 0.10-0.20 158 32 0.16 0.14-0.19
Malignant 806 161 0.06 0.06-0.06 70 14 0.03 0.03-0.04 -- -- -- -- 36 7 0.04 0.03-0.05
Non-Malignant 3,258 652 0.28 0.27-0.29 444 89 0.19 0.17-0.21 28 6 0.11 0.07-0.16 122 24 0.13 0.10-0.15
Tumors of the Pineal Region 622 124 0.05 0.05-0.05 111 22 0.05 0.04-0.06 -- -- -- -- 22 4 0.02 0.01-0.04
Malignant 331 66 0.03 0.02-0.03 79 16 0.03 0.03-0.04 -- -- -- -- -- -- -- --
Non-Malignant 291 58 0.02 0.02-0.03 32 6 0.01 0.01-0.02 -- -- -- -- -- -- -- --
Embryonal Tumors 2,745 549 0.25 0.24-0.26 399 80 0.17 0.15-0.18 35 7 0.14 0.10-0.19 130 26 0.15 0.12-0.17
Tumors of Cranial and Spinal Nerves 30,265 6,053 2.13 2.11-2.16 2,241 448 1.05 1.01-1.10 227 45 1.11 0.97-1.27 1,662 332 1.66 1.58-1.75
Nerve Sheath Tumors 30,231 6,046 2.13 2.11-2.16 2,238 448 1.05 1.00-1.09 226 45 1.11 0.96-1.27 1,661 332 1.66 1.58-1.75
Malignant 171 34 0.01 0.01-0.01 27 5 0.01 0.01-0.02 -- -- -- -- -- -- -- --
Non-Malignant 30,060 6,012 2.12 2.09-2.14 2,211 442 1.04 0.99-1.08 225 45 1.10 0.96-1.26 1,647 329 1.65 1.57-1.73
Other Tumors of Cranial and Spinal Nerves 34 7 0.00 0.00-0.00 -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 132,895 26,579 8.90 8.85-8.95 20,560 4,112 10.44 10.29-10.59 964 193 5.70 5.32-6.09 5,871 1,174 6.49 6.33-6.67
Meningioma 129,114 25,823 8.61 8.56-8.66 20,081 4,016 10.22 10.08-10.37 939 188 5.59 5.22-5.98 5,709 1,142 6.33 6.16-6.50
Malignant 1,368 274 0.09 0.09-0.10 261 52 0.13 0.12-0.15 -- -- -- -- 82 16 0.09 0.07-0.11
Non-Malignant 127,746 25,549 8.52 8.47-8.57 19,820 3,964 10.09 9.95-10.24 -- -- -- -- 5,627 1,125 6.24 6.07-6.41
Mesenchymal Tumors 1,216 243 0.09 0.09-0.10 144 29 0.07 0.06-0.08 -- -- -- -- 60 12 0.06 0.05-0.08
Primary Melanocytic Lesions 99 20 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Other Neoplasms Related to the Meninges 2,466 493 0.19 0.18-0.19 330 66 0.15 0.13-0.17 19 4 0.08 0.05-0.13 100 20 0.10 0.08-0.12
Lymphoma and Hematopoietic Neoplasms 6,854 1,371 0.45 0.44-0.46 653 131 0.32 0.29-0.34 51 10 0.29 0.21-0.39 417 83 0.45 0.41-0.50
Lymphoma 6,678 1,336 0.44 0.43-0.45 619 124 0.30 0.28-0.33 48 10 0.28 0.20-0.37 405 81 0.44 0.39-0.48
Other Hematopoietic Neoplasms 176 35 0.01 0.01-0.02 34 7 0.02 0.01-0.02 -- -- -- -- -- -- -- --
Germ Cell Tumors and Cysts 1,238 248 0.11 0.10-0.11 167 33 0.07 0.06-0.08 -- -- -- -- 118 24 0.13 0.11-0.15
Germ Cell Tumors, Cysts and Heterotopias 1,238 248 0.11 0.10-0.11 167 33 0.07 0.06-0.08 -- -- -- -- 118 24 0.13 0.11-0.15
Malignant 840 168 0.08 0.07-0.08 113 23 0.05 0.04-0.06 -- -- -- -- 100 20 0.11 0.09-0.13
Non-Malignant 398 80 0.03 0.03-0.04 54 11 0.02 0.02-0.03 -- -- -- -- 18 4 0.02 0.01-0.03
Tumors of Sellar Region 52,691 10,538 3.97 3.93-4.00 14,603 2,921 7.00 6.88-7.11 629 126 3.10 2.85-3.36 2,913 583 2.96 2.85-3.07
Tumors of the Pituitary 50,437 10,087 3.79 3.76-3.83 13,964 2,793 6.70 6.59-6.82 603 121 2.97 2.73-3.23 2,784 557 2.82 2.72-2.93
Malignant 102 20 0.01 0.01-0.01 28 6 0.01 0.01-0.02 -- -- -- -- -- -- -- --
Non-Malignant 50,335 10,067 3.78 3.75-3.82 13,936 2,787 6.69 6.57-6.80 600 120 2.96 2.72-3.22 2,781 556 2.82 2.71-2.93
Craniopharyngioma 2,254 451 0.18 0.17-0.18 639 128 0.29 0.27-0.32 26 5 0.12 0.08-0.19 129 26 0.14 0.11-0.16
Unclassified Tumors 16,694 3,339 1.17 1.16-1.19 2,151 430 1.09 1.04-1.14 158 32 0.89 0.75-1.05 576 115 0.65 0.60-0.71
Hemangioma 4,739 948 0.36 0.35-0.37 562 112 0.27 0.24-0.29 61 12 0.30 0.23-0.39 216 43 0.22 0.19-0.26
Neoplasm Unspecified 11,823 2,365 0.81 0.79-0.82 1,547 309 0.80 0.76-0.84 96 19 0.58 0.46-0.72 349 70 0.41 0.37-0.46
Malignant 5,697 1,139 0.37 0.36-0.38 568 114 0.31 0.28-0.33 47 9 0.29 0.21-0.39 159 32 0.20 0.17-0.23
Non-Malignant 6,126 1,225 0.43 0.42-0.45 979 196 0.49 0.46-0.53 49 10 0.29 0.21-0.39 190 38 0.22 0.19-0.25
All Other 132 26 0.01 0.01-0.01 42 8 0.02 0.01-0.03 -- -- -- -- -- -- -- --
TOTALd 339,661 67,932 23.83 23.75-23.91 48,876 9,775 23.88 23.67-24.10 2,670 534 14.23 13.67-14.82 14,118 2,824 15.04 14.78-15.29
Malignant 107,737 21,547 7.58 7.54-7.63 9,432 1,886 4.44 4.34-4.53 692 138 3.54 3.26-3.83 3,169 634 3.38 3.27-3.51
Non-Malignant 231,924 46,385 16.25 16.18-16.32 39,444 7,889 19.45 19.25-19.65 1,978 396 10.69 10.20-11.21 10,949 2,190 11.65 11.43-11.88

a. Annual average cases are calculated by dividing the five- year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Individuals with unknown race were excluded (N = 10,086).

d. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

  • Incidence rates for all primary brain and other CNS tumors combined were lower for race-groups AIAN (14.23 per 100,000 population) compared to Whites (23.83 per 100,000 population), Blacks (23.88 per 100,000 population), and API (15.04 per 100,000 population).

  • Incidence rates for non-malignant primary brain and other CNS tumors were highest in Blacks (19.45 per 100,000) compared to Whites (16.25 per 100,000), AIAN (10.69 per 100,000), and API (11.65 per 100,000).

  • Incidence rates for malignant primary brain and other CNS tumors were highest in Whites (7.58 per 100,000) compared to Blacks (4.44 per 100,000), AIAN (3.54 per 100,000), and API (3.38 per 100,000).

  • Incidence rates of meningioma, tumors of the pituitary, and craniopharyngioma observed for Blacks exceeded those observed for Whites, AIAN, and API.

Average annual age-adjusted incidence rates and incidence rate-ratios (White: Black) for selected histologies are presented in Fig. 15 and Fig. 16, respectively.

Fig. 15.

Fig. 15

Average Annual Age-Adjusted Incidence Ratesa with 95% Confidence Intervals of Selected Primary Brain and Other CNS Tumor Histologies by Race, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Fig. 16.

Fig. 16

Incidence Rate Ratios by Race (Whites:Blacks and Whites:Asian Or Pacific Islanders [API]) for Selected Primary Brain and Other CNS Tumor Histologies, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • Though overall incidence of primary brain and other CNS tumor was slightly higher in Blacks as compared to Whites, incidence of many specific histologies was significantly higher among Whites.

  • Incidence rates for glioblastoma (p<0.0001), all other astrocytoma (p<0.0001), and nerve sheath tumors (p<0.0001) were approximately 2 times greater in Whites than in Blacks.

  • Incidence of oligodendroglioma was 2.36 times greater in Whites than in Blacks (p<0.0001).

  • Incidence rates for pilocytic astrocytoma (p<0.0001), ependymal tumors (p<0.0001), embryonal tumors (p<0.0001), lymphoma (p<0.0001), and germ cell tumors (p<0.0001) were also higher among Whites than Blacks.

  • Incidence rates for both non-malignant (p<0.0001) and malignant (p<0.0001) meningioma and tumors of the pituitary (p<0.0001) were higher among Blacks than Whites.

Average annual age-adjusted incidence rates and incidence rate ratios (White:API) for selected histologies are presented in Fig. 15 and Fig. 16, respectively.

  • Incidence of glioblastoma (p<0.0001) was 2.97 times greater in Whites than in API.

  • Incidence of nerve sheath tumors (p<0.0001) was 1.28 times higher in Whites than in API.

Incidence Rates by Hispanic Ethnicity and Histology

Incidence rates by Hispanic ethnicity and histology are presented in Table 13, and incidence rate-ratios (Non-Hispanic:Hispanic) for selected histologies are presented in Supplementary Figure 12.

Table 13.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Hispanic Ethnicityc, and Race, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology All Hispanic White Hispanic Black Hispanic All Non-Hispanic
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Tumors of Neuroepithelial Tissue 11,766 2,353 5.01 4.92-5.11 10,815 2,163 5.10 5.00-5.20 291 58 2.60 2.27-2.97 101,233 20,247 6.85 6.81-6.89
Pilocytic Astrocytoma 812 162 0.24 0.23-0.26 732 146 0.25 0.23-0.27 22 4 0.12 0.07-0.19 4,345 869 0.38 0.37-0.40
Diffuse Astrocytoma 816 163 0.33 0.30-0.35 751 150 0.33 0.31-0.36 17 3 0.12 0.06-0.20 6,606 1,321 0.48 0.47-0.49
Anaplastic Astrocytoma 665 133 0.28 0.26-0.30 611 122 0.28 0.26-0.31 21 4 0.17 0.10-0.27 6,447 1,289 0.45 0.44-0.46
Unique Astrocytoma Variants 189 38 0.07 0.06-0.08 173 35 0.07 0.06-0.08 -- -- -- -- 934 187 0.07 0.07-0.08
Malignant 113 23 0.04 0.03-0.05 106 21 0.04 0.04-0.05 -- -- -- -- 656 131 0.05 0.05-0.05
Non-Malignant 76 15 0.02 0.02-0.03 67 13 0.02 0.02-0.03 -- -- -- -- 278 56 0.02 0.02-0.03
Glioblastoma 4,723 945 2.46 2.39-2.53 4,414 883 2.51 2.44-2.59 118 24 1.41 1.15-1.71 55,322 11,064 3.32 3.29-3.35
Oligodendroglioma 429 86 0.16 0.15-0.18 395 79 0.17 0.15-0.19 -- -- -- -- 3,265 653 0.25 0.24-0.26
Anaplastic Oligodendroglioma 235 47 0.10 0.08-0.11 214 43 0.10 0.08-0.11 -- -- -- -- 1,624 325 0.12 0.11-0.12
Oligoastrocytic Tumors 170 34 0.07 0.06-0.08 158 32 0.07 0.06-0.08 -- -- -- -- 1,401 280 0.11 0.10-0.11
Ependymal Tumors 962 192 0.36 0.34-0.39 868 174 0.36 0.34-0.39 20 4 0.15 0.08-0.24 5,876 1,175 0.44 0.42-0.45
Malignant 624 125 0.23 0.21-0.25 570 114 0.23 0.21-0.25 -- -- -- -- 3,346 669 0.25 0.24-0.26
Non-Malignant 338 68 0.13 0.12-0.15 298 60 0.13 0.12-0.15 -- -- -- -- 2,530 506 0.18 0.18-0.19
Glioma Malignant, NOS 1,042 208 0.40 0.37-0.42 934 187 0.40 0.37-0.43 38 8 0.29 0.19-0.42 7,047 1,409 0.54 0.53-0.55
Choroid Plexus Tumors 164 33 0.05 0.04-0.06 152 30 0.05 0.05-0.06 -- -- -- -- 662 132 0.05 0.05-0.06
Malignant 26 5 0.01 0.00-0.01 24 5 0.01 0.00-0.01 -- -- -- -- 102 20 0.01 0.01-0.01
Non-Malignant 138 28 0.05 0.04-0.05 128 26 0.05 0.04-0.06 -- -- -- -- 560 112 0.05 0.04-0.05
Other Neuroepithelial Tumors 21 4 0.01 0.00-0.01 17 3 0.01 0.00-0.01 -- -- -- -- 86 17 0.01 0.01-0.01
Malignant -- -- -- -- -- -- -- -- -- -- -- -- 52 10 0.00 0.00-0.01
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- 34 7 0.00 0.00-0.00
Neuronal and Mixed Neuronal Glial Tumors 677 135 0.23 0.21-0.24 605 121 0.23 0.21-0.24 16 3 0.08 0.05-0.14 4,250 850 0.34 0.33-0.35
Malignant 123 25 0.05 0.04-0.06 110 22 0.05 0.04-0.06 -- -- -- -- 823 165 0.06 0.05-0.06
Non-Malignant 554 111 0.18 0.16-0.19 495 99 0.18 0.16-0.20 -- -- -- -- 3,427 685 0.28 0.27-0.29
Tumors of the Pineal Region 116 23 0.04 0.03-0.05 107 21 0.04 0.03-0.05 -- -- -- -- 670 134 0.05 0.05-0.06
Malignant 76 15 0.03 0.02-0.03 70 14 0.03 0.02-0.03 -- -- -- -- 370 74 0.03 0.03-0.03
Non-Malignant 40 8 0.02 0.01-0.02 37 7 0.02 0.01-0.02 -- -- -- -- 300 60 0.02 0.02-0.03
Embryonal Tumors 745 149 0.22 0.21-0.24 684 137 0.23 0.21-0.25 -- -- -- -- 2,698 540 0.24 0.23-0.25
Tumors of Cranial and Spinal Nerves 3,193 639 1.41 1.36-1.46 2,873 575 1.40 1.35-1.45 81 16 0.72 0.56-0.91 32,378 6,476 2.14 2.11-2.16
Nerve Sheath Tumors 3,187 637 1.41 1.36-1.46 2,867 573 1.40 1.34-1.45 81 16 0.72 0.56-0.91 32,344 6,469 2.13 2.11-2.16
Malignant 43 9 0.02 0.01-0.03 37 7 0.02 0.01-0.03 -- -- -- -- 180 36 0.01 0.01-0.01
Non-Malignant 3,144 629 1.39 1.34-1.44 2,830 566 1.38 1.33-1.43 81 16 0.72 0.56-0.91 32,164 6,433 2.12 2.10-2.15
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- -- 34 7 0.00 0.00-0.00
Tumors of Meninges 15,869 3,174 8.44 8.30-8.58 14,496 2,899 8.43 8.28-8.57 457 91 5.62 5.07-6.20 147,677 29,535 9.21 9.16-9.26
Meningioma 15,254 3,051 8.20 8.06-8.34 13,927 2,785 8.18 8.04-8.32 450 90 5.57 5.02-6.15 143,716 28,743 8.93 8.88-8.98
Malignant 187 37 0.09 0.08-0.11 173 35 0.10 0.08-0.11 -- -- -- -- 1,563 313 0.10 0.09-0.10
Non-Malignant 15,067 3,013 8.10 7.97-8.24 13,754 2,751 8.08 7.94-8.22 443 89 5.48 4.94-6.05 142,153 28,431 8.83 8.79-8.88
Mesenchymal Tumors 211 42 0.08 0.07-0.10 198 40 0.09 0.08-0.10 -- -- -- -- 1,250 250 0.09 0.09-0.10
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- -- -- -- -- 98 20 0.01 0.01-0.01
Other Neoplasms Related to the Meninges 394 79 0.16 0.14-0.17 362 72 0.16 0.14-0.18 -- -- -- -- 2,613 523 0.18 0.18-0.19
Lymphoma and Hematopoietic Neoplasms 956 191 0.50 0.47-0.53 896 179 0.51 0.48-0.55 18 4 0.17 0.09-0.27 7,192 1,438 0.44 0.43-0.45
Lymphoma 924 185 0.49 0.45-0.52 868 174 0.50 0.47-0.54 18 4 0.17 0.09-0.27 6,993 1,399 0.43 0.42-0.44
Other Hematopoietic Neoplasms 32 6 0.01 0.01-0.02 28 6 0.01 0.01-0.02 -- -- -- -- 199 40 0.01 0.01-0.02
Germ Cell Tumors and Cysts 347 69 0.11 0.10-0.12 314 63 0.11 0.10-0.12 -- -- -- -- 1,237 247 0.11 0.10-0.11
Germ Cell Tumors, Cysts and Heterotopias 347 69 0.11 0.10-0.12 314 63 0.11 0.10-0.12 -- -- -- -- 1,237 247 0.11 0.10-0.11
Malignant 251 50 0.07 0.07-0.08 226 45 0.08 0.07-0.09 -- -- -- -- 843 169 0.07 0.07-0.08
Non-Malignant 96 19 0.03 0.03-0.04 88 18 0.03 0.03-0.04 -- -- -- -- 394 79 0.03 0.03-0.04
Tumors of Sellar Region 11,655 2,331 4.86 4.77-4.95 10,443 2,089 4.82 4.72-4.91 400 80 3.54 3.17-3.94 61,618 12,324 4.32 4.29-4.36
Tumors of the Pituitary 11,184 2,237 4.68 4.59-4.77 10,011 2,002 4.64 4.54-4.73 386 77 3.45 3.08-3.85 58,960 11,792 4.13 4.09-4.16
Malignant 25 5 0.01 0.01-0.02 21 4 0.01 0.01-0.02 -- -- -- -- 117 23 0.01 0.01-0.01
Non-Malignant 11,159 2,232 4.67 4.58-4.76 9,990 1,998 4.63 4.53-4.72 386 77 3.45 3.08-3.85 58,843 11,769 4.12 4.08-4.15
Craniopharyngioma 471 94 0.18 0.16-0.19 432 86 0.18 0.16-0.20 -- -- -- -- 2,658 532 0.20 0.19-0.21
Unclassified Tumors 2,394 479 1.15 1.10-1.20 2,187 437 1.16 1.11-1.22 63 13 0.65 0.48-0.86 17,658 3,532 1.16 1.15-1.18
Hemangioma 850 170 0.35 0.33-0.38 782 156 0.36 0.33-0.39 21 4 0.16 0.09-0.26 4,876 975 0.35 0.34-0.36
Neoplasm Unspecified 1,524 305 0.79 0.75-0.83 1,389 278 0.79 0.75-0.84 42 8 0.49 0.34-0.69 12,608 2,522 0.81 0.79-0.82
Malignant 600 120 0.35 0.32-0.38 555 111 0.35 0.32-0.38 -- -- -- -- 5,987 1,197 0.36 0.36-0.37
Non-Malignant 924 185 0.44 0.41-0.47 834 167 0.44 0.41-0.47 -- -- -- -- 6,621 1,324 0.44 0.43-0.45
All Other 20 4 0.01 0.01-0.02 16 3 0.01 0.01-0.02 -- -- -- -- 174 35 0.01 0.01-0.01
TOTAL d 46,180 9,236 21.48 21.27-21.69 42,024 8,405 21.53 21.31-21.75 1,321 264 13.36 12.57-14.18 368,993 73,799 24.23 24.15-24.31
Malignant 12,782 2,556 5.70 5.60-5.81 11,795 2,359 5.81 5.70-5.93 308 62 2.83 2.48-3.21 110,653 22,131 7.33 7.29-7.37
Non-Malignant 33,398 6,680 15.78 15.60-15.96 30,229 6,046 15.72 15.53-15.90 1,013 203 10.54 9.83-11.28 258,340 51,668 16.90 16.84-16.97

a. Annual average cases are calculated by dividing the five year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Hispanic ethnicity is not mutually exclusive of race; Classified using the North American Association of Central Cancer Registries Hispanic Identification Algorithm, version 2 (NHIA v2).

d. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

  • The overall incidence rate for primary brain and other CNS tumors was 21.48 per 100,000 population among Hispanics and 24.23 per 100,000 population among non-Hispanics.

  • Tumors of the pituitary and lymphoma were the only histologies that were higher in Hispanics than in non-Hispanics.

While there are several histologies where significant differences in incidence were observed by race and/or ethnicity, in most cases the actual difference in incidence rates is small and may not be biologically significant.

Incidence and Distribution of Primary Brain and Other CNS Tumors in Childhood and Adolescence by Site, Histology, Sex, and Age

Distribution of Tumors by Site and Histology in Children and Adolescents (Age 0-19 Years)

Brain and other CNS tumors are the most common form of solid tumors in children and account for the majority of cancer mortality in this age group. About 6% of the reported brain and other CNS tumors during 2013-2017 occurred in children and adolescents age 0-19 years. The distribution of brain and other CNS tumors for children and adolescents age 0-19 years by site is presented in Fig. 17A.

Fig. 17.

Fig. 17

Distributiona in Children and Adolescents (Age 0-19 Years) of Primary Brain and CNS Tumors (Malignant and Non-Malignant Combined; Five-Year Total=25,105; Annual Average Cases=5,021) by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • The largest percentages of tumors in childhood and adolescence were located in the Pituitary and Craniopharyngeal duct (17%).

  • Frontal, temporal, parietal, and occipital lobes of the brain accounted for 6%, 6.7%, 2.6%, and 1.2% of all brain and other CNS tumors in childhood and adolescence, respectively.

  • Cerebrum, ventricle, brain stem, and cerebellum tumors accounted for 5.3%, 5.2%, 10.9%, and 13% of all brain and other CNS tumors in childhood and adolescence, respectively.

  • The cranial nerves and the spinal cord and cauda equina accounted for 7.2% and 5.1% of all brain and other CNS tumors in childhood and adolescence, respectively.

The most common brain and other CNS histologies in children and adolescents age 0-19 years are presented in Fig. 17B.

  • For children and adolescents age 0-19 years, pilocytic astrocytoma, glioma malignant, NOS, and embryonal tumors accounted for 14.9%, 11.9%, and 9.9%, respectively.

  • Tumors of the pituitary were the most common non-glial and predominantly non-malignant histology and accounted for 13.5% of all tumors in this age group.

  • Gliomas accounted for approximately 45.5% of tumors in children and adolescents age 0-19 years.

  • Medulloblastoma accounted for 65.8% of all embryonal tumors in this age group.

Distribution of Tumors by Site and Histology in Children (Age 0-14 Years)

Approximately 4.3% of all reported tumors occurred in children age 0-14 years. The distribution of brain and other CNS tumors for children age 0-14 years is presented by site and histology in Fig. 18A and Fig. 18B, respectively.

Fig. 18.

Fig. 18

Distributiona in Children (Age 0-14 Years) of Primary Brain and Other CNS Tumors (Malignant and Non-Malignant Combined; Five-Year Total=17,673; Annual Average Cases=3,535) by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2012-2016

  • Tumors of cerebellum (15.3%) comprised the largest proportion of tumors followed by the other brain (14.2%) and brain stem (13.3%).

  • For children age 0-14 years, pilocytic astrocytoma, glioma malignant, NOS, and embryonal tumors accounted for 17.7%, 14.5%, and 12.7%, respectively.

  • Gliomas accounted for approximately 51.6% of tumors in children age 0-14 years.

  • Of embryonal tumors, medulloblastoma, atypical teratoid rhabdoid tumor (ATRT), and primitive neuroectodermal tumor (PNET) accounted for 64.7%, 16.6%, and 9.5%, respectively.

Distribution of Tumors by Site and Histology in Adolescents (Age 15-19 Years)

About 1.8% of the reported brain and other CNS tumors during 2013-2017 occurred in adolescents age 15-19 years for a total of 7,432 tumors diagnosed between 2013 and 2017 (Table 6). The distribution of these tumors by site and histology is presented in Fig. 19A and Fig. 19B, respectively.

Table 6.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Children and Adolescents (Age 0-19 Years), Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and Age at Diagnosis, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology Age at Diagnosis
0-19 Years 0-4 Years 5-9 Years 10-14 Years 15-19 Years
5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI 5-Year Total Annual Average Rate 95% CI
Tumors of Neuroepithelial Tissue 15,802 3,160 3.88 3.82-3.94 4,875 975 4.93 4.79-5.07 4,097 819 4.03 3.91-4.16 3,747 749 3.65 3.53-3.77 3,083 617 2.94 2.84-3.05
Pilocytic Astrocytoma 3,744 749 0.92 0.89-0.95 1,148 230 1.16 1.09-1.23 1,059 212 1.04 0.98-1.11 921 184 0.90 0.84-0.96 616 123 0.59 0.54-0.64
Diffuse Astrocytoma 954 191 0.23 0.22-0.25 263 53 0.27 0.23-0.30 190 38 0.19 0.16-0.22 250 50 0.24 0.21-0.28 251 50 0.24 0.21-0.27
Anaplastic Astrocytoma 394 79 0.10 0.09-0.11 66 13 0.07 0.05-0.08 107 21 0.11 0.09-0.13 110 22 0.11 0.09-0.13 111 22 0.11 0.09-0.13
Unique Astrocytoma Variants 490 98 0.12 0.11-0.13 103 21 0.10 0.09-0.13 127 25 0.12 0.10-0.15 149 30 0.15 0.12-0.17 111 22 0.11 0.09-0.13
Malignant 237 47 0.06 0.05-0.07 19 4 0.02 0.01-0.03 52 10 0.05 0.04-0.07 87 17 0.08 0.07-0.10 79 16 0.08 0.06-0.09
Non-Malignant 253 51 0.06 0.05-0.07 84 17 0.09 0.07-0.11 75 15 0.07 0.06-0.09 62 12 0.06 0.05-0.08 32 6 0.03 0.02-0.04
Glioblastoma 717 143 0.18 0.16-0.19 110 22 0.11 0.09-0.13 169 34 0.17 0.14-0.19 201 40 0.20 0.17-0.22 237 47 0.23 0.20-0.26
Oligodendroglioma 175 35 0.04 0.04-0.05 19 4 0.02 0.01-0.03 25 5 0.02 0.02-0.04 42 8 0.04 0.03-0.06 89 18 0.08 0.07-0.10
Anaplastic Oligodendroglioma 27 5 0.01 0.00-0.01 -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Oligoastrocytic Tumors 64 13 0.02 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- -- 26 5 0.02 0.02-0.04
Ependymal Tumors 1,191 238 0.29 0.28-0.31 457 91 0.46 0.42-0.51 242 48 0.24 0.21-0.27 260 52 0.25 0.22-0.29 232 46 0.22 0.19-0.25
Malignant 997 199 0.24 0.23-0.26 439 88 0.44 0.40-0.49 215 43 0.21 0.18-0.24 191 38 0.19 0.16-0.21 152 30 0.14 0.12-0.17
Non-Malignant 194 39 0.05 0.04-0.05 18 4 0.02 0.01-0.03 27 5 0.03 0.02-0.04 69 14 0.07 0.05-0.09 80 16 0.08 0.06-0.09
Glioma Malignant, NOS 2,989 598 0.73 0.71-0.76 930 186 0.94 0.88-1.00 967 193 0.95 0.89-1.01 661 132 0.64 0.60-0.69 431 86 0.41 0.37-0.45
Choroid Plexus Tumors 409 82 0.10 0.09-0.11 258 52 0.26 0.23-0.30 54 11 0.05 0.04-0.07 53 11 0.05 0.04-0.07 44 9 0.04 0.03-0.06
Malignant 104 21 0.03 0.02-0.03 85 17 0.09 0.07-0.11 -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 305 61 0.07 0.07-0.08 173 35 0.18 0.15-0.20 -- -- -- -- -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 32 6 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 1,927 385 0.47 0.45-0.49 331 66 0.34 0.30-0.37 357 71 0.35 0.32-0.39 610 122 0.59 0.55-0.64 629 126 0.60 0.55-0.65
Malignant 111 22 0.03 0.02-0.03 23 5 0.02 0.01-0.03 20 4 0.02 0.01-0.03 27 5 0.03 0.02-0.04 41 8 0.04 0.03-0.05
Non-Malignant 1,816 363 0.44 0.42-0.47 308 62 0.31 0.28-0.35 337 67 0.33 0.30-0.37 583 117 0.57 0.52-0.62 588 118 0.56 0.52-0.61
Tumors of the Pineal Region 211 42 0.05 0.04-0.06 60 12 0.06 0.05-0.08 48 10 0.05 0.03-0.06 45 9 0.04 0.03-0.06 58 12 0.06 0.04-0.07
Malignant 174 35 0.04 0.04-0.05 -- -- -- -- -- -- -- -- -- -- -- -- 41 8 0.04 0.03-0.05
Non-Malignant 37 7 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- -- 17 3 0.02 0.01-0.03
Embryonal Tumors 2,478 496 0.61 0.58-0.63 1,106 221 1.12 1.05-1.19 727 145 0.72 0.66-0.77 419 84 0.41 0.37-0.45 226 45 0.22 0.19-0.25
Medulloblastoma 1,637 327 0.40 0.38-0.42 492 98 0.50 0.45-0.54 619 124 0.61 0.56-0.66 352 70 0.34 0.31-0.38 174 35 0.17 0.14-0.19
Primitive neuroectodermal tumors 241 48 0.06 0.05-0.07 129 26 0.13 0.11-0.16 50 10 0.05 0.04-0.06 35 7 0.03 0.02-0.05 27 5 0.03 0.02-0.04
Atypical teratoid rhabdoid tumor 380 76 0.09 0.08-0.10 325 65 0.33 0.29-0.37 33 7 0.03 0.02-0.05 16 3 0.02 0.01-0.03 -- -- -- --
Other embryonal histologies 220 44 0.05 0.05-0.06 160 32 0.16 0.14-0.19 25 5 0.02 0.02-0.04 16 3 0.02 0.01-0.03 19 4 0.02 0.01-0.03
Tumors of Cranial and Spinal Nerves 1,291 258 0.32 0.30-0.33 290 58 0.29 0.26-0.33 246 49 0.24 0.21-0.27 304 61 0.30 0.26-0.33 451 90 0.43 0.39-0.47
Nerve Sheath Tumors 1,288 258 0.31 0.30-0.33 290 58 0.29 0.26-0.33 246 49 0.24 0.21-0.27 304 61 0.30 0.26-0.33 448 90 0.43 0.39-0.47
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 1,133 227 0.28 0.26-0.29 204 41 0.21 0.18-0.24 134 27 0.13 0.11-0.16 254 51 0.25 0.22-0.28 541 108 0.52 0.47-0.56
Meningioma 649 130 0.16 0.15-0.17 72 14 0.07 0.06-0.09 77 15 0.08 0.06-0.09 157 31 0.15 0.13-0.18 343 69 0.33 0.29-0.36
Malignant 34 7 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 615 123 0.15 0.14-0.16 -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Mesenchymal Tumors 267 53 0.07 0.06-0.07 125 25 0.13 0.11-0.15 50 10 0.05 0.04-0.06 48 10 0.05 0.03-0.06 44 9 0.04 0.03-0.06
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Other Neoplasms Related to the Meninges 205 41 0.05 0.04-0.06 -- -- -- -- -- -- -- -- 46 9 0.04 0.03-0.06 150 30 0.14 0.12-0.17
Lymphoma and Hematopoietic Neoplasms 122 24 0.03 0.02-0.04 17 3 0.02 0.01-0.03 33 7 0.03 0.02-0.05 28 6 0.03 0.02-0.04 44 9 0.04 0.03-0.06
Lymphoma 59 12 0.01 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- -- --- -- -- --
Other Hematopoietic Neoplasms 63 13 0.02 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Germ Cell Tumors and Cysts 993 199 0.24 0.23-0.26 184 37 0.19 0.16-0.22 165 33 0.16 0.14-0.19 345 69 0.34 0.30-0.37 299 60 0.29 0.25-0.32
Germ Cell Tumors, Cysts and Heterotopias 993 199 0.24 0.23-0.26 184 37 0.19 0.16-0.22 165 33 0.16 0.14-0.19 345 69 0.34 0.30-0.37 299 60 0.29 0.25-0.32
Malignant 775 155 0.19 0.18-0.20 80 16 0.08 0.06-0.10 120 24 0.12 0.10-0.14 308 62 0.30 0.27-0.34 267 53 0.25 0.22-0.29
Non-Malignant 218 44 0.05 0.05-0.06 104 21 0.11 0.09-0.13 45 9 0.04 0.03-0.06 37 7 0.04 0.03-0.05 32 6 0.03 0.02-0.04
Tumors of Sellar Region 4,254 851 1.03 1.00-1.07 197 39 0.20 0.17-0.23 610 122 0.60 0.55-0.65 908 182 0.88 0.83-0.94 2,539 508 2.42 2.33-2.52
Tumors of the Pituitary 3,393 679 0.82 0.79-0.85 40 8 0.04 0.03-0.06 304 61 0.30 0.27-0.33 685 137 0.67 0.62-0.72 2,364 473 2.25 2.16-2.35
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Craniopharyngioma 861 172 0.21 0.20-0.23 157 31 0.16 0.13-0.19 306 61 0.30 0.27-0.34 223 45 0.22 0.19-0.25 175 35 0.17 0.14-0.19
Unclassified Tumors 1,510 302 0.37 0.35-0.39 338 68 0.34 0.31-0.38 300 60 0.30 0.26-0.33 397 79 0.39 0.35-0.43 475 95 0.45 0.41-0.50
Hemangioma 598 120 0.15 0.13-0.16 126 25 0.13 0.11-0.15 101 20 0.10 0.08-0.12 147 29 0.14 0.12-0.17 224 45 0.21 0.19-0.24
Neoplasm Unspecified 872 174 0.21 0.20-0.23 195 39 0.20 0.17-0.23 192 38 0.19 0.16-0.22 241 48 0.23 0.21-0.27 244 49 0.23 0.20-0.26
Malignant 218 44 0.05 0.05-0.06 73 15 0.07 0.06-0.09 51 10 0.05 0.04-0.07 40 8 0.04 0.03-0.05 54 11 0.05 0.04-0.07
Non-Malignant 654 131 0.16 0.15-0.17 122 24 0.12 0.10-0.15 141 28 0.14 0.12-0.16 201 40 0.20 0.17-0.22 190 38 0.18 0.16-0.21
All Other 40 8 0.01 0.01-0.01 17 3 0.02 0.01-0.03 -- -- -- -- -- -- -- -- -- -- -- --
TOTAL c 25,105 5,021 6.14 6.07-6.22 6,105 1,221 6.18 6.02-6.33 5,585 1,117 5.49 5.35-5.64 5,983 1,197 5.83 5.68-5.98 7,432 1,486 7.09 6.93-7.25
Malignant 14,463 2,893 3.55 3.49-3.61 4,498 900 4.55 4.42-4.68 3,847 769 3.78 3.67-3.91 3,393 679 3.31 3.19-3.42 2,725 545 2.60 2.50-2.70
Non-Malignant 10,642 2,128 2.60 2.55-2.65 1,607 321 1.63 1.55-1.71 1,738 348 1.71 1.63-1.79 2,590 518 2.52 2.43-2.62 4,707 941 4.49 4.36-4.62

a. Annual average cases are calculated by dividing the five-year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Refers to all brain tumors including histologies not presented in this table.

-- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

Fig. 19.

Fig. 19

Distributiona in Adolescents (Age 15-19 Years) of Primary Brain and Other CNS Tumors (Malignant and Non-Malignant Combined; Five-Year Total=7,432; Annual Average Cases=1,486) by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • 34.6% of these tumors were diagnosed in the pituitary and craniopharyngeal duct.

  • The frontal lobe, temporal lobe, occipital lobe, and parietal lobe accounted for 19.2% of tumors in this age group.

  • The most common histology in adolescents was tumors of the pituitary (31.8%).

  • Gliomas accounted for approximately 31.1% of tumors in adolescents. Of these gliomas, the histology pilocytic astrocytoma accounted for 8.3% of all tumors in this age group.

Incidence Rates by Histology, Histology Groupings, and Sex in Children and Adolescents (Age 0-19 Years)

The incidence rates of the most common brain and other CNS tumors in children and adolescents by major histology grouping, histology, and sex are presented in Table 14.

Table 14.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Children and Adolescents (Age 0-19 Years), Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and Sex, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology Total Male Female
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Tumors of Neuroepithelial Tissue 15,802 3,160 3.88 3.82-3.94 8,556 1,711 4.11 4.02-4.20 7,246 1,449 3.63 3.55-3.72
Pilocytic Astrocytoma 3,744 749 0.92 0.89-0.95 1,959 392 0.94 0.90-0.98 1,785 357 0.90 0.86-0.94
Diffuse Astrocytoma 954 191 0.23 0.22-0.25 513 103 0.25 0.23-0.27 441 88 0.22 0.20-0.24
Anaplastic Astrocytoma 394 79 0.10 0.09-0.11 217 43 0.10 0.09-0.12 177 35 0.09 0.08-0.10
Unique Astrocytoma Variants 490 98 0.12 0.11-0.13 270 54 0.13 0.11-0.15 220 44 0.11 0.10-0.13
Malignant 237 47 0.06 0.05-0.07 129 26 0.06 0.05-0.07 108 22 0.05 0.04-0.07
Non-Malignant 253 51 0.06 0.05-0.07 141 28 0.07 0.06-0.08 112 22 0.06 0.05-0.07
Glioblastoma 717 143 0.18 0.16-0.19 397 79 0.19 0.17-0.21 320 64 0.16 0.14-0.18
Oligodendroglioma 175 35 0.04 0.04-0.05 87 17 0.04 0.03-0.05 88 18 0.04 0.04-0.05
Anaplastic Oligodendroglioma 27 5 0.01 0.00-0.01 -- -- -- -- -- -- -- --
Oligoastrocytic Tumors 64 13 0.02 0.01-0.02 32 6 0.02 0.01-0.02 32 6 0.02 0.01-0.02
Ependymal Tumors 1,191 238 0.29 0.28-0.31 668 134 0.32 0.30-0.35 523 105 0.26 0.24-0.29
Malignant 997 199 0.24 0.23-0.26 558 112 0.27 0.25-0.29 439 88 0.22 0.20-0.24
Non-Malignant 194 39 0.05 0.04-0.05 110 22 0.05 0.04-0.06 84 17 0.04 0.03-0.05
Glioma Malignant, NOS 2,989 598 0.73 0.71-0.76 1,506 301 0.72 0.69-0.76 1,483 297 0.74 0.71-0.78
Choroid Plexus Tumors 409 82 0.10 0.09-0.11 231 46 0.11 0.10-0.13 178 36 0.09 0.08-0.10
Malignant 104 21 0.03 0.02-0.03 60 12 0.03 0.02-0.04 44 9 0.02 0.02-0.03
Non-Malignant 305 61 0.07 0.07-0.08 171 34 0.08 0.07-0.10 134 27 0.07 0.06-0.08
Other Neuroepithelial Tumors 32 6 0.01 0.01-0.01 -- -- -- -- -- -- -- --
Malignant -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 1,927 385 0.47 0.45-0.49 1,063 213 0.51 0.48-0.54 864 173 0.43 0.40-0.46
Malignant 111 22 0.03 0.02-0.03 64 13 0.03 0.02-0.04 47 9 0.02 0.02-0.03
Non-Malignant 1,816 363 0.44 0.42-0.47 999 200 0.48 0.45-0.51 817 163 0.41 0.38-0.44
Tumors of the Pineal Region 211 42 0.05 0.04-0.06 106 21 0.05 0.04-0.06 105 21 0.05 0.04-0.06
Malignant 174 35 0.04 0.04-0.05 -- -- -- -- -- -- -- --
Non-Malignant 37 7 0.01 0.01-0.01 -- -- -- -- -- -- -- --
Embryonal Tumors 2,478 496 0.61 0.58-0.63 1,481 296 0.71 0.68-0.75 997 199 0.50 0.47-0.53
Medulloblastoma 1,637 327 0.40 0.38-0.42 1,055 211 0.51 0.48-0.54 582 116 0.29 0.27-0.32
Primitive neuroectodermal tumors 241 48 0.06 0.05-0.07 121 24 0.06 0.05-0.07 120 24 0.06 0.05-0.07
Atypical teratoid rhabdoid tumor 380 76 0.09 0.08-0.10 197 39 0.09 0.08-0.11 183 37 0.09 0.08-0.11
Other embryonal histologies 220 44 0.05 0.05-0.06 108 22 0.05 0.04-0.06 112 22 0.06 0.05-0.07
Tumors of Cranial and Spinal Nerves 1,291 258 0.32 0.30-0.33 687 137 0.33 0.30-0.35 604 121 0.30 0.28-0.33
Nerve Sheath Tumors 1,288 258 0.31 0.30-0.33 684 137 0.33 0.30-0.35 604 121 0.30 0.28-0.33
Malignant -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- --
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 1,133 227 0.28 0.26-0.29 539 108 0.26 0.24-0.28 594 119 0.30 0.27-0.32
Meningioma 649 130 0.16 0.15-0.17 292 58 0.14 0.12-0.16 357 71 0.18 0.16-0.20
Malignant 34 7 0.01 0.01-0.01 -- -- -- -- -- -- -- --
Non-Malignant 615 123 0.15 0.14-0.16 -- -- -- -- -- -- -- --
Mesenchymal Tumors 267 53 0.07 0.06-0.07 143 29 0.07 0.06-0.08 124 25 0.06 0.05-0.07
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- -- -- -- --
Other Neoplasms Related to the Meninges 205 41 0.05 0.04-0.06 97 19 0.05 0.04-0.06 108 22 0.05 0.04-0.06
Lymphoma and Hematopoietic Neoplasms 122 24 0.03 0.02-0.04 76 15 0.04 0.03-0.05 46 9 0.02 0.02-0.03
Lymphoma 59 12 0.01 0.01-0.02 40 8 0.02 0.01-0.03 19 4 0.01 0.01-0.01
Other Hematopoietic Neoplasms 63 13 0.02 0.01-0.02 36 7 0.02 0.01-0.02 27 5 0.01 0.01-0.02
Germ Cell Tumors and Cysts 993 199 0.24 0.23-0.26 671 134 0.32 0.30-0.35 322 64 0.16 0.14-0.18
Germ Cell Tumors, Cysts and Heterotopias 993 199 0.24 0.23-0.26 671 134 0.32 0.30-0.35 322 64 0.16 0.14-0.18
Malignant 775 155 0.19 0.18-0.20 550 110 0.26 0.24-0.29 225 45 0.11 0.10-0.13
Non-Malignant 218 44 0.05 0.05-0.06 121 24 0.06 0.05-0.07 97 19 0.05 0.04-0.06
Tumors of Sellar Region 4,254 851 1.03 1.00-1.07 1,374 275 0.66 0.62-0.69 2,880 576 1.43 1.38-1.48
Tumors of the Pituitary 3,393 679 0.82 0.79-0.85 915 183 0.44 0.41-0.46 2,478 496 1.23 1.18-1.28
Malignant -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- --
Craniopharyngioma 861 172 0.21 0.20-0.23 459 92 0.22 0.20-0.24 402 80 0.20 0.18-0.22
Unclassified Tumors 1,510 302 0.37 0.35-0.39 757 151 0.36 0.34-0.39 753 151 0.38 0.35-0.40
Hemangioma 598 120 0.15 0.13-0.16 302 60 0.14 0.13-0.16 296 59 0.15 0.13-0.17
Neoplasm Unspecified 872 174 0.21 0.20-0.23 436 87 0.21 0.19-0.23 436 87 0.22 0.20-0.24
Malignant 218 44 0.05 0.05-0.06 113 23 0.05 0.04-0.07 105 21 0.05 0.04-0.06
Non-Malignant 654 131 0.16 0.15-0.17 323 65 0.16 0.14-0.17 331 66 0.17 0.15-0.18
All Other 40 8 0.01 0.01-0.01 19 4 0.01 0.01-0.01 21 4 0.01 0.01-0.02
TOTAL c 25,105 5,021 6.14 6.07-6.22 12,660 2,532 6.07 5.96-6.18 12,445 2,489 6.22 6.11-6.33
Malignant 14,463 2,893 3.55 3.49-3.61 7,943 1,589 3.81 3.73-3.90 6,520 1,304 3.27 3.19-3.35
Non-Malignant 10,642 2,128 2.60 2.55-2.65 4,717 943 2.26 2.19-2.32 5,925 1,185 2.95 2.88-3.03

a. Annual average cases are calculated by dividing the five year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

  • Average annual incidence rates were highest for tumors of neuroepithelial tissue (3.88 per 100,000 population). Among these tumors, the most common histologies were pilocytic astrocytoma (0.92 per 100,000 population), glioma malignant, NOS (0.73 per 100,000 population), and embryonal tumors (0.61 per 100,000 population).

  • There were notable differences in incidence rates between males and females for ependymal tumors, embryonal tumors, germ cell tumors, and tumors of the pituitary.

Incidence Rates by Histology and Race/Ethnicity in Children and Adolescents (Age 0-19 Years)

Incidence rates for brain and other CNS tumors by histology and race for children and adolescents age 0-19 years are presented in Table 15 and by race/ethnicity in Table 16.

Table 15.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Children and Adolescents (Age 0-19 Years), Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, and Racec, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology White Black American Indian/Alaska Native Asian/Pacific Islander
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Tumors of Neuroepithelial Tissue 12,466 2,493 4.06 3.99-4.13 2,030 406 2.97 2.85-3.11 135 27 1.78 1.50-2.11 512 102 2.05 1.88-2.24
Pilocytic Astrocytoma 3,014 603 0.98 0.95-1.02 457 91 0.67 0.61-0.73 28 6 0.37 0.25-0.53 89 18 0.36 0.29-0.44
Diffuse Astrocytoma 754 151 0.25 0.23-0.26 113 23 0.17 0.14-0.20 -- -- -- -- 33 7 0.13 0.09-0.19
Anaplastic Astrocytoma 311 62 0.10 0.09-0.11 50 10 0.07 0.05-0.10 -- -- -- -- 19 4 0.08 0.05-0.12
Unique Astrocytoma Variants 373 75 0.12 0.11-0.13 73 15 0.11 0.08-0.14 -- -- -- -- 17 3 0.07 0.04-0.11
Malignant 195 39 0.06 0.05-0.07 24 5 0.04 0.02-0.05 -- -- -- -- -- -- -- --
Non-Malignant 178 36 0.06 0.05-0.07 49 10 0.07 0.05-0.10 -- -- -- -- -- -- -- --
Glioblastoma 550 110 0.18 0.16-0.19 94 19 0.14 0.11-0.17 -- -- -- -- 35 7 0.14 0.10-0.20
Oligodendroglioma 137 27 0.04 0.04-0.05 23 5 0.03 0.02-0.05 -- -- -- -- -- -- -- --
Anaplastic Oligodendroglioma 18 4 0.01 0.00-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Oligoastrocytic Tumors 54 11 0.02 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- --
Ependymal Tumors 960 192 0.31 0.29-0.33 144 29 0.21 0.18-0.25 -- -- -- -- 43 9 0.17 0.12-0.23
Malignant 791 158 0.26 0.24-0.28 -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 169 34 0.05 0.05-0.06 -- -- -- -- -- -- -- -- -- -- -- --
Glioma Malignant, NOS 2,331 466 0.76 0.73-0.79 417 83 0.61 0.55-0.67 24 5 0.31 0.20-0.47 82 16 0.33 0.26-0.41
Choroid Plexus Tumors 327 65 0.11 0.10-0.12 50 10 0.07 0.05-0.10 -- -- -- -- -- -- -- --
Malignant 78 16 0.03 0.02-0.03 16 3 0.02 0.01-0.04 -- -- -- -- -- -- -- --
Non-Malignant 249 50 0.08 0.07-0.09 34 7 0.05 0.03-0.07 -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 24 5 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 1,537 307 0.50 0.47-0.52 247 49 0.36 0.32-0.41 16 3 0.21 0.12-0.35 59 12 0.24 0.18-0.31
Malignant 86 17 0.03 0.02-0.03 18 4 0.03 0.02-0.04 -- -- -- -- -- -- -- --
Non-Malignant 1,451 290 0.47 0.45-0.50 229 46 0.34 0.29-0.38 -- -- -- -- -- -- -- --
Tumors of the Pineal Region 140 28 0.05 0.04-0.05 49 10 0.07 0.05-0.10 -- -- -- -- -- -- -- --
Malignant 109 22 0.04 0.03-0.04 -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 31 6 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Embryonal Tumors 1,936 387 0.63 0.61-0.66 298 60 0.43 0.39-0.49 26 5 0.34 0.22-0.49 111 22 0.44 0.36-0.53
Medulloblastoma 1,300 260 0.43 0.40-0.45 173 35 0.25 0.22-0.29 18 4 0.24 0.14-0.37 77 15 0.31 0.24-0.38
Primitive neuroectodermal tumors 183 37 0.06 0.05-0.07 39 8 0.06 0.04-0.08 -- -- -- -- -- -- -- --
Atypical teratoid rhabdoid tumor 280 56 0.09 0.08-0.10 59 12 0.08 0.06-0.11 -- -- -- -- 19 4 0.08 0.05-0.12
Other embryonal histologies 173 35 0.06 0.05-0.07 27 5 0.04 0.03-0.06 -- -- -- -- -- -- -- --
Tumors of Cranial and Spinal Nerves 1,002 200 0.32 0.30-0.35 162 32 0.24 0.20-0.28 -- -- -- -- 44 9 0.18 0.13-0.24
Nerve Sheath Tumors 999 200 0.32 0.30-0.34 162 32 0.24 0.20-0.28 -- -- -- -- 44 9 0.18 0.13-0.24
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- 44 9 0.18 0.13-0.24
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 894 179 0.29 0.27-0.31 150 30 0.22 0.19-0.26 -- -- -- -- 33 7 0.13 0.09-0.19
Meningioma 506 101 0.16 0.15-0.18 94 19 0.14 0.11-0.17 -- -- -- -- 19 4 0.08 0.05-0.12
Malignant 28 6 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 478 96 0.15 0.14-0.17 -- -- -- -- -- -- -- -- 19 4 0.08 0.05-0.12
Mesenchymal Tumors 209 42 0.07 0.06-0.08 32 6 0.05 0.03-0.07 -- -- -- -- -- -- -- --
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Other Neoplasms Related to the Meninges 168 34 0.05 0.05-0.06 24 5 0.04 0.02-0.05 -- -- -- -- -- -- -- --
Lymphoma and Hematopoietic Neoplasms 89 18 0.03 0.02-0.04 -- -- -- -- -- -- -- -- -- -- -- --
Lymphoma 42 8 0.01 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- --
Other Hematopoietic Neoplasms 47 9 0.02 0.01-0.02 -- -- -- -- -- -- -- -- -- -- -- --
Germ Cell Tumors and Cysts 756 151 0.25 0.23-0.26 106 21 0.16 0.13-0.19 -- -- -- -- 87 17 0.35 0.28-0.43
Germ Cell Tumors, Cysts and Heterotopias 756 151 0.25 0.23-0.26 106 21 0.16 0.13-0.19 -- -- -- -- 87 17 0.35 0.28-0.43
Malignant 588 118 0.19 0.18-0.21 79 16 0.12 0.09-0.15 -- -- -- -- -- -- -- --
Non-Malignant 168 34 0.05 0.05-0.06 27 5 0.04 0.03-0.06 -- -- -- -- -- -- -- --
Tumors of Sellar Region 3,170 634 1.02 0.98-1.05 657 131 0.96 0.89-1.04 59 12 0.80 0.61-1.03 145 29 0.59 0.49-0.69
Tumors of the Pituitary 2,536 507 0.81 0.78-0.84 500 100 0.73 0.67-0.80 53 11 0.72 0.54-0.94 106 21 0.43 0.35-0.52
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- 500 100 0.73 0.67-0.80 -- -- -- -- 106 21 0.43 0.35-0.52
Craniopharyngioma 634 127 0.21 0.19-0.22 157 31 0.23 0.20-0.27 -- -- -- -- 39 8 0.16 0.11-0.21
Unclassified Tumors 1,193 239 0.39 0.37-0.41 178 36 0.26 0.22-0.30 17 3 0.23 0.13-0.36 34 7 0.14 0.09-0.19
Hemangioma 496 99 0.16 0.15-0.18 51 10 0.07 0.06-0.10 -- -- -- -- 18 4 0.07 0.04-0.11
Neoplasm Unspecified 671 134 0.22 0.20-0.24 115 23 0.17 0.14-0.20 -- -- -- -- 16 3 0.06 0.04-0.10
Malignant 159 32 0.05 0.04-0.06 37 7 0.05 0.04-0.07 -- -- -- -- -- -- -- --
Non-Malignant 512 102 0.17 0.15-0.18 78 16 0.11 0.09-0.14 -- -- -- -- -- -- -- --
All Other 26 5 0.01 0.01-0.01 -- -- -- -- -- -- -- -- -- -- -- --
TOTAL d 19,570 3,914 6.36 6.27-6.45 3,294 659 4.83 4.66-4.99 242 48 3.22 2.83-3.65 868 174 3.48 3.26-3.72
Malignant 11,343 2,269 3.70 3.63-3.77 1,857 371 2.72 2.60-2.85 131 26 1.73 1.45-2.05 529 106 2.12 1.94-2.31
Non-Malignant 8,227 1,645 2.66 2.60-2.72 1,437 287 2.11 2.00-2.22 111 22 1.49 1.23-1.80 339 68 1.36 1.22-1.52

a. Annual average cases are calculated by dividing the five year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Individuals with unknown race were excluded (N = 630).

d. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified

Table 16.

Five-Year Total, Annual Average Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Children and Adolescents (Age 0-19 Years), Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Hispanic Ethnicityc, and Race, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Histology All Hispanic White Hispanic Black Hispanic All Non-Hispanic
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Tumors of Neuroepithelial Tissue 2,977 595 2.96 2.85-3.07 2,704 541 3.04 2.93-3.16 82 16 1.34 1.06-1.66 12,805 2,561 4.17 4.09-4.24
Pilocytic Astrocytoma 651 130 0.64 0.60-0.70 591 118 0.66 0.61-0.72 -- -- -- -- 3,084 617 1.01 0.97-1.04
Diffuse Astrocytoma 147 29 0.15 0.12-0.17 134 27 0.15 0.13-0.18 -- -- -- -- 807 161 0.26 0.24-0.28
Anaplastic Astrocytoma 85 17 0.09 0.07-0.11 76 15 0.09 0.07-0.11 -- -- -- -- 309 62 0.10 0.09-0.11
Unique Astrocytoma Variants 108 22 0.11 0.09-0.13 97 19 0.11 0.09-0.13 -- -- -- -- 382 76 0.12 0.11-0.14
Malignant 48 10 0.05 0.04-0.07 45 9 0.05 0.04-0.07 -- -- -- -- 189 38 0.06 0.05-0.07
Non-Malignant 60 12 0.06 0.05-0.08 52 10 0.06 0.04-0.08 -- -- -- -- 193 39 0.06 0.05-0.07
Glioblastoma 152 30 0.15 0.13-0.18 143 29 0.16 0.14-0.19 -- -- -- -- 565 113 0.18 0.17-0.20
Oligodendroglioma 21 4 0.02 0.01-0.03 20 4 0.02 0.01-0.04 -- -- -- -- 154 31 0.05 0.04-0.06
Anaplastic Oligodendroglioma -- -- -- -- -- -- -- -- -- -- -- -- 20 4 0.01 0.00-0.01
Oligoastrocytic Tumors -- -- -- -- -- -- -- -- -- -- -- -- 54 11 0.02 0.01-0.02
Ependymal Tumors 255 51 0.25 0.22-0.29 236 47 0.27 0.23-0.30 -- -- -- -- 936 187 0.30 0.29-0.32
Malignant 220 44 0.22 0.19-0.25 205 41 0.23 0.20-0.26 -- -- -- -- 777 155 0.25 0.24-0.27
Non-Malignant 35 7 0.04 0.03-0.05 31 6 0.04 0.02-0.05 -- -- -- -- 159 32 0.05 0.04-0.06
Glioma Malignant, NOS 515 103 0.51 0.47-0.56 456 91 0.51 0.47-0.56 21 4 0.33 0.20-0.51 2,470 494 0.81 0.78-0.84
Choroid Plexus Tumors 101 20 0.10 0.08-0.12 93 19 0.10 0.08-0.13 -- -- -- -- 307 61 0.10 0.09-0.11
Malignant 23 5 0.02 0.01-0.03 21 4 0.02 0.01-0.04 -- -- -- -- 81 16 0.03 0.02-0.03
Non-Malignant 78 16 0.08 0.06-0.10 72 14 0.08 0.06-0.10 -- -- -- -- 226 45 0.07 0.06-0.08
Other Neuroepithelial Tumors -- -- -- -- -- -- -- -- -- -- -- -- 23 5 0.01 0.00-0.01
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 327 65 0.33 0.30-0.37 294 59 0.34 0.30-0.38 -- -- -- -- 1,596 319 0.51 0.49-0.54
Malignant 24 5 0.02 0.02-0.04 22 4 0.03 0.02-0.04 -- -- -- -- 87 17 0.03 0.02-0.03
Non-Malignant 303 61 0.31 0.27-0.34 272 54 0.31 0.28-0.35 -- -- -- -- 1,509 302 0.49 0.46-0.51
Tumors of the Pineal Region 44 9 0.04 0.03-0.06 38 8 0.04 0.03-0.06 -- -- -- -- 167 33 0.05 0.05-0.06
Malignant -- -- -- -- -- -- -- -- -- -- -- -- 139 28 0.05 0.04-0.05
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- 28 6 0.01 0.01-0.01
Embryonal Tumors 545 109 0.53 0.49-0.58 503 101 0.56 0.51-0.61 -- -- -- -- 1,931 386 0.63 0.61-0.66
Medulloblastoma 350 70 0.35 0.31-0.38 326 65 0.36 0.33-0.41 -- -- -- -- 1,286 257 0.42 0.40-0.45
Primitive neuroectodermal tumors 58 12 0.06 0.04-0.07 53 11 0.06 0.04-0.08 -- -- -- -- 183 37 0.06 0.05-0.07
Atypical teratoid rhabdoid tumor 85 17 0.08 0.06-0.10 78 16 0.08 0.07-0.11 -- -- -- -- 295 59 0.10 0.09-0.11
Other embryonal histologies 52 10 0.05 0.04-0.07 46 9 0.05 0.04-0.07 -- -- -- -- 167 33 0.05 0.05-0.06
Tumors of Cranial and Spinal Nerves 243 49 0.25 0.22-0.28 213 43 0.24 0.21-0.28 -- -- -- -- 1,046 209 0.34 0.32-0.36
Nerve Sheath Tumors 242 48 0.24 0.21-0.28 212 42 0.24 0.21-0.28 -- -- -- -- 1,044 209 0.34 0.32-0.36
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- -
Other Tumors of Cranial and Spinal Nerves -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Tumors of Meninges 244 49 0.25 0.22-0.28 221 44 0.26 0.22-0.29 -- -- -- -- 887 177 0.28 0.26-0.30
Meningioma 125 25 0.13 0.11-0.15 112 22 0.13 0.11-0.16 -- -- -- -- 523 105 0.17 0.15-0.18
Malignant -- -- -- -- -- -- -- -- -- -- -- -- 26 5 0.01 0.01-0.01
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- -- 497 99 0.16 0.14-0.17
Mesenchymal Tumors 66 13 0.07 0.05-0.08 60 12 0.07 0.05-0.09 -- -- -- -- 200 40 0.07 0.06-0.07
Primary Melanocytic Lesions -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Other Neoplasms Related to the Meninges 50 10 0.05 0.04-0.07 47 9 0.06 0.04-0.07 -- -- -- -- 155 31 0.05 0.04-0.06
Lymphoma and Hematopoietic Neoplasms 22 4 0.02 0.01-0.03 19 4 0.02 0.01-0.03 -- -- -- -- 100 20 0.03 0.03-0.04
Lymphoma -- -- -- -- -- -- -- -- -- -- -- -- 46 9 0.01 0.01-0.02
Other Hematopoietic Neoplasms -- -- -- -- -- -- -- -- -- -- -- -- 54 11 0.02 0.01-0.02
Germ Cell Tumors and Cysts 246 49 0.25 0.22-0.28 224 45 0.26 0.22-0.29 -- -- -- -- 746 149 0.24 0.22-0.26
Germ Cell Tumors, Cysts and Heterotopias 246 49 0.25 0.22-0.28 224 45 0.26 0.22-0.29 -- -- -- -- 746 149 0.24 0.22-0.26
Malignant 195 39 0.20 0.17-0.23 177 35 0.21 0.18-0.24 -- -- -- -- 579 116 0.19 0.17-0.20
Non-Malignant 51 10 0.05 0.04-0.07 47 9 0.05 0.04-0.07 -- -- -- -- 167 33 0.05 0.05-0.06
Tumors of Sellar Region 1,139 228 1.19 1.12-1.26 1,024 205 1.20 1.13-1.28 34 7 0.60 0.42-0.84 3,108 622 0.99 0.95-1.02
Tumors of the Pituitary 951 190 1.00 0.94-1.07 847 169 1.00 0.94-1.07 -- -- -- -- 2,435 487 0.77 0.74-0.80
Malignant -- -- -- -- -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant -- -- -- -- -- -- -- -- -- -- -- - -- -- -- --
Craniopharyngioma 188 38 0.19 0.16-0.22 177 35 0.20 0.17-0.23 -- -- -- -- 673 135 0.22 0.20-0.24
Unclassified Tumors 335 67 0.34 0.30-0.38 291 58 0.33 0.30-0.37 -- -- -- -- 1,174 235 0.38 0.36-0.40
Hemangioma 135 27 0.14 0.11-0.16 124 25 0.14 0.12-0.17 -- -- -- -- 463 93 0.15 0.14-0.16
Neoplasm Unspecified 194 39 0.20 0.17-0.23 161 32 0.18 0.16-0.22 -- -- -- -- 677 135 0.22 0.20-0.24
Malignant 50 10 0.05 0.04-0.07 42 8 0.05 0.03-0.06 -- -- -- -- 168 34 0.05 0.05-0.06
Non-Malignant 144 29 0.15 0.12-0.17 119 24 0.14 0.11-0.16 -- -- -- -- 509 102 0.16 0.15-0.18
All Other -- -- -- -- -- -- -- -- -- -- -- -- 34 7 0.01 0.01-0.02
TOTAL d 5,206 1,041 5.26 5.11-5.40 4,696 939 5.36 5.21-5.52 148 30 2.47 2.09-2.90 19,866 3,973 6.42 6.33-6.51
Malignant 2,797 559 2.78 2.68-2.88 2,544 509 2.86 2.75-2.98 76 15 1.23 0.97-1.54 11,650 2,330 3.79 3.72-3.86
Non-Malignant 2,409 482 2.48 2.38-2.58 2,152 430 2.50 2.39-2.61 72 14 1.24 0.97-1.56 8,216 1,643 2.63 2.57-2.69

a. Annual average cases are calculated by dividing the five year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Hispanic ethnicity is not mutually exclusive of race; Classified using the North American Association of Central Cancer Registries Hispanic Identification Algorithm, version 2 (NHIA v2).

d. Refers to all brain tumors including histologies not presented in this table.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program; CI, confidence interval; NOS, not otherwise specified.

  • Incidence rates were highest among White (6.36 per 100,000 population) compared to Blacks (4.83 per 100,000 population), AIAN (3.22 per 100,000 population), and API (3.48 per 100,000 population).

Incidence Rates by Age and Histology in Children and Adolescents (Age 0-19 Years)

Detailed age-adjusted incidence rates for brain and other CNS tumors by histology for, children and adolescents age 0-19 years overall, and age groups 0-4 years, 5-9 years, 10-14 years, and 15-19 years are presented in Table 6.

  • Overall, incidence rates for age groups 0-4 years (6.18 per 100,000 population) and 15-19 years (7.09 per 100,000 population) exceeded those observed in age groups 5-9 years (5.49 per 100,000 population) and 10-14 years (5.83 per 100,000 population).

  • Individual histology distributions varied substantially within these age groups.

  • Incidence rates of pilocytic astrocytoma, glioma malignant, NOS, ependymal tumors, choroid plexus tumors, and embryonal tumors decreased with increasing age.

Incidence Rates by Histology Defined by ICCC in Children and Adolescents (Age 0-19 Years)

The CBTRUS brain and other CNS tumor data for children and adolescents used for this report according to the International Classification of Childhood Cancer (ICCC) grouping system for pediatric cancers are presented in Supplementary Table 8 (See Supplementary Table 1 for more additional information on the ICCC classification scheme).

Incidence and Distribution of Primary Brain and Other CNS Tumors in Adolescent and Young Adults (Age 15-39 Years)

About 14.5% of the reported brain and other CNS tumors during 2013-2017 occurred in adolescents and young adults age 15-39 years for a total of 60,358 tumors diagnosed between 2013 and 2017 (Table 5). The distribution of these tumors by site and histology is presented in Fig. 23A and Fig. 23B, respectively.

Fig. 23.

Fig. 23

Distributiona of Primary Spinal Cord, Spinal Meninges, and Cauda Equina Tumors by Histology in A) Children and Adolescents (Age 0-19 Years, Five-Year Total=1,371; Annual Average Cases=274) and B) Adults (Age 20+ Years, Five-Year Total=18,502; Annual Average Cases=3,700), CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

  • The overall incidence rate in this age group was 11.54 per 100,000 population (Table 5). Incidence of malignant tumors was 3.23 per 100,000, and incidence of non-malignant tumors was 8.31 per 100,000.

  • Tumors of the sellar region had the highest incidence (4.07 per 100,000 population), followed by tumors of the neuroepithelial tissue (3.46 per 100,000 population) (Table 5).

  • The most common histology in AYA was tumors of the pituitary (3.94 per 100,000 population), followed by meningioma (1.89 per 100,000 population) and nerve sheath tumors (1.05 per 100,000 population) (Table 5).

  • The majority of AYA brain and other CNS tumors occurred in the pituitary and craniopharyngeal duct (36.1%), followed by the meninges (15.9%) (Fig. 20A).

  • Approximately 17.6% of tumors diagnosed in AYA were located within the frontal, temporal, parietal, and occipital lobes of the brain combined (Fig. 20A).

  • Cerebrum, ventricle, cerebellum, and brain stem tumors combined accounted for about 6.3% of all AYA tumors (Fig. 20A).

  • The predominately non-malignant tumors of the pituitary (28%), meningioma (12.5%), and nerve sheath (8%) represented over half of CNS tumors diagnosed in AYA (Fig. 20B).

  • Glioma accounted for approximately 25.6% of all brain and other CNS tumors in AYA, and about 82.4% of all malignant tumors (Fig. 20B).

Fig. 20.

Fig. 20

Distributiona in Adolescents and Young Adultsb (Age 15-39 Years) of Primary Brain and Other CNS Tumors (Five-Year Total=60,358; Annual Average Cases=12,072) by A) Site and B) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Estimated Numbers of Expected Cases Primary Brain and Other CNS Tumors

Estimated Numbers of Expected Cases of All Primary Brain and Other CNS Tumors by State

The estimated number of cases of all primary brain and other CNS tumors for 2020 and 2021 by State and Behavior are presented in Table 17. Overall totals presented are based on total malignant and nonmalignant incidence, and it should be noted that these presented stratified rates may not add up to these totals. Estimated numbers of cases are highly dependent on input data. Different patterns of incidence within strata can substantively affect the projected estimates, and strata-specific estimates may not equal the total estimate presented. Therefore, caution should be used when utilizing these estimates.

Table 17.

Estimated Number of Casesa,b of Brain and Other Central Nervous System Tumors Overall and by Behavior by State, 2020, 2021

State 2020 Estimated Cases 2021 Estimated Cases
All Malignant Non-Malignant All Malignant Non-Malignant
Alabama 1,090 390 700 1,110 390 720
Alaska 190 60 130 200 60 130
Arizona 1,510 490 1,020 1,500 490 1,010
Arkansas 810 280 530 830 280 540
California 8,720 2,870 5,850 8,620 2,900 5,720
Colorado 1,750 450 1,300 1,800 460 1,340
Connecticut 1,010 310 700 1,030 310 720
Delaware 190 90 110 190 90 100
District of Columbia 170 -- -- 170 -- --
Florida 6,670 1,790 4,890 6,800 1,800 4,990
Georgia 3,410 730 2,690 3,570 730 2,840
Hawaii 320 80 240 320 80 240
Idaho 480 150 330 500 150 340
Illinois 3,380 1,020 2,360 3,360 1,030 2,330
Indiana 1,050 550 500 970 550 420
Iowa 1,020 280 740 1,050 280 760
Kansas 800 230 570 820 230 590
Kentucky 1,390 450 940 1,390 460 930
Louisiana 1,540 350 1,190 1,600 350 1,250
Maine 310 140 170 310 140 170
Maryland 1,750 460 1,290 1,820 460 1,350
Massachusetts 1,660 580 1,080 1,690 590 1,110
Michigan 2,410 800 1,610 2,410 800 1,610
Minnesota 1,620 510 1,110 1,710 520 1,190
Mississippi 800 230 570 820 230 590
Missouri 1,740 530 1,220 1,770 530 1,230
Montana 330 100 230 340 100 240
Nebraska 480 170 310 490 170 320
Nevada 770 260 510 810 270 540
New Hampshire 400 140 260 410 140 260
New Jersey 2,290 760 1,520 2,220 770 1,450
New Mexico 480 150 330 490 150 340
New York 6,870 1,690 5,180 7,030 1,710 5,330
North Carolina 3,000 860 2,140 3,060 870 2,190
North Dakota 200 60 140 200 60 150
Ohio 2,510 1,040 1,470 2,460 1,050 1,410
Oklahoma 710 310 410 690 310 380
Oregon 1,010 390 620 1,030 390 630
Pennsylvania 4,150 1,230 2,920 4,190 1,240 2,950
Rhode Island 220 80 130 220 80 130
South Carolina 1,090 440 660 1,050 450 600
South Dakota 240 70 170 250 70 180
Tennessee 1,820 510 1,310 1,820 510 1,320
Texas 7,310 2,030 5,270 7,430 2,070 5,370
Utah 1,460 230 1,230 1,580 240 1,340
Vermont 190 60 130 190 60 130
Virginia 2,300 690 1,610 2,430 700 1,730
Washington 2,980 670 2,300 3,090 690 2,410
West Virginia 520 130 390 520 120 400
Wisconsin 1,780 440 1,340 1,790 440 1,360
Wyoming 160 50 110 170 50 120

a. Source: Estimation based on CBTRUS NPCR and SEER 2000-2017 data for malignant tumors, and NPCR and SEER 2006-2017 data for non-malignant tumors.

b. Rounded to the nearest 10. Numbers may not add up due to rounding.

- Estimated number is less than 50. These cases are included in overall rates.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program

Estimated Number of Expected Cases of All Primary Brain and Other CNS Tumors by Histology, Histology Grouping, and Age

The estimated number of cases of all primary brain and other CNS tumors for 2020 and 2021 by histology and age are presented in Table 18 and Supplementary Table 10. Overall totals presented are based on total malignant and non-malignant incidence, and it should be noted that these presented stratified rates may not add up to these totals. Estimated numbers of cases are highly dependent on input data. Different patterns of incidence within strata can substantively affect the projected estimates, and strata-specific estimates may not equal the total estimate presented. Therefore, caution should be used when utilizing these estimates.

Table 18.

Estimated Number of Casesa,b of Brain and Other Central Nervous System Tumors Overall and by Behavior, by Major Histology Groupingc, and Histology, 2020, 2021

Histology 2020 Estimated Cases 2021 Estimated Cases
All Malignant Non-Malignant All Malignant Non-Malignant
Tumors of Neuroepithelial Tissue 23,110 21,370 1,740 23,300 21,530 1,770
Pilocytic Astrocytoma 960 960 -- 950 950 --
Diffuse Astrocytoma 1,420 1,420 -- 1,410 1,410 --
Anaplastic Astrocytoma 1,350 1,350 -- 1,340 1,340 --
Unique Astrocytoma Variants 130 100 -- 120 90 --
Glioblastoma 12,800 12,800 -- 12,970 12,970 --
Oligodendroglioma 730 730 -- 730 730 --
Anaplastic Oligodendroglioma 420 420 -- 430 430 --
Oligoastrocytic Tumors -- -- -- -- -- --
Ependymal Tumors 1,310 760 540 1,290 760 540
Glioma Malignant, NOS 1,920 1,920 -- 1,990 1,990 --
Choroid Plexus Tumors 150 -- 140 150 -- 130
Other Neuroepithelial Tumors -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 1,110 140 960 1,130 130 1,000
Tumors of the Pineal Region 130 80 -- 120 80 --
Embryonal Tumors 650 640 -- 640 630 --
Tumors of Cranial and Spinal Nerves 5,910 -- 5,870 5,670 -- 5,630
Nerve Sheath Tumors 5,910 -- 5,870 5,670 -- 5,630
Tumors of Meninges 35,260 400 34,850 35,800 390 35,410
Meningioma 34,300 240 34,060 34,840 220 34,620
Mesenchymal Tumors 330 120 210 340 130 220
Primary Melanocytic Lesions -- -- -- -- -- --
Other Neoplasms Related to the Meninges 600 -- -- 600 -- --
Lymphomas and Hematopoietic Neoplasms 1,810 1,810 -- 1,850 1,850 --
Lymphoma 1,770 1,770 -- 1,820 1,820 --
Other Hematopoietic Neoplasms -- -- -- -- -- --
Germ Cell Tumors and Cysts 350 260 90 360 270 90
Germ Cell Tumors, Cysts and Heterotopias 350 260 90 360 270 90
Tumors of Sellar Region 14,430 -- 14,420 14,370 -- 14,360
Tumors of the Pituitary 13,830 -- 13,820 13,770 -- 13,760
Craniopharyngioma 600 -- 600 600 -- 600
Unclassified Tumors 2,950 1,080 1,870 2,810 1,040 1,770
Hemangioma 700 -- 690 640 -- 630
Neoplasm Unspecified 2,100 1,040 1,050 1,970 990 980
All Other 150 -- 120 200 -- 170
TOTAL 83,830 24,970 58,860 84,170 25,130 59,040

a. Source: Estimation based on CBTRUS NPCR and SEER 2000-2017 data for malignant tumors, and NPCR and SEER 2006-2017 data for non-malignant tumors.

b. Rounded to the nearest 10. Numbers may not add up due to rounding.

c. Total estimate is based on overall estimate. Histology-specific estimates may not add up to total.

- Estimated number is less than 50. These cases are included in overall rates.

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; SEER, Surveillance, Epidemiology, and End Results Program

  • The total number of new cases of primary brain and other CNS tumors in 2020 is estimated to be 83,830, with 24,970 malignant and 58,860 non-malignant cases.

  • For 2021, the estimate is 84,170 new cases of primary brain and other CNS tumors of which 25,130 and 59,040 are expected to be malignant and non-malignant, respectively.

  • Meningiomas have the highest number of all estimated new cases, with 34,300 cases projected in 2020 and 34,840 in 2021

  • Glioblastoma has the highest number of cases of all malignant tumors, with 12,800 cases projected in 2020 and 12,970 in 2021.

  • For 2020, the highest number of new cases is predicted in those age 65+ years, with 38,000 cases. For 2021, the highest number of new cases is estimated to be in those age 65+ years, with 38,900 cases.

  • For 2020 and 2021, children age 0-14 years are estimated to have 3,440 and 3,460 new cases of primary brain and other CNS tumors each year, respectively.

  • For 2020 and 2021, children age 0-19 years are estimated to have 4,620 and 4,630 new cases of primary brain and other CNS tumors each year, respectively.

  • AYA are estimated to have 11,720 new primary brain and other CNS tumors in 2020 and 11,700 in 2021 (Supplementary Table 10).

Mortality Rates

Mortality Rates for Malignant Brain and Other CNS Tumors by State, Sex, and Urban/Rural Residence

AAAMR for primary malignant brain and other CNS tumors in the US during 2013-2017 by state and sex are presented in Table 19 and Fig. 21. AAAMR for primary malignant brain and other CNS tumors by state and urban/rural residence are presented in Supplementary Table 11.

Table 19.

Five-Year Total, Average Annual Totala, and Average Annual Age-Adjusted Mortality Ratesb for Malignant Brain and Other Central Nervous System Cancer Overall and by State and Sex, United States, 2013-2017c

State Total Male Female
5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI 5 year total Annual average Rate 95% CI
Alabama 1,476 295 5.12 4.86-5.40 819 164 6.25 5.81-6.70 657 131 4.20 3.87-4.54
Alaska 147 29 4.18 3.49-4.97 74 15 4.08 3.12-5.24 73 15 4.26 3.29-5.42
Arizona 1,708 342 4.27 4.07-4.48 963 193 5.10 4.78-5.44 745 149 3.53 3.27-3.80
Arkansas 873 175 4.94 4.61-5.28 492 98 6.10 5.56-6.68 381 76 3.97 3.57-4.41
California 8,972 1,794 4.34 4.25-4.43 5,084 1,017 5.32 5.17-5.47 3,888 778 3.50 3.39-3.61
Colorado 1,279 256 4.37 4.13-4.63 691 138 4.98 4.60-5.38 588 118 3.84 3.53-4.17
Connecticut 945 189 4.34 4.06-4.64 540 108 5.44 4.98-5.93 405 81 3.43 3.09-3.80
Delaware 252 50 4.27 3.75-4.86 137 27 5.14 4.29-6.11 115 23 3.60 2.95-4.36
Washington DC 78 16 2.28 1.80-2.87 48 10 3.12 2.28-4.17 30 6 1.61 1.08-2.34
Florida 5,635 1,127 4.18 4.07-4.30 3,157 631 5.09 4.91-5.27 2,478 496 3.38 3.24-3.52
Georgia 2,275 455 4.27 4.09-4.45 1,269 254 5.22 4.92-5.52 1,006 201 3.47 3.26-3.70
Hawaii 253 51 2.92 2.56-3.32 140 28 3.43 2.87-4.07 113 23 2.43 1.99-2.95
Idaho 480 96 5.13 4.67-5.63 306 61 6.93 6.16-7.78 174 35 3.53 3.01-4.11
Illinois 3,037 607 4.18 4.03-4.33 1,691 338 5.11 4.86-5.36 1,346 269 3.40 3.22-3.60
Indiana 1,705 341 4.55 4.34-4.78 987 197 5.68 5.32-6.06 718 144 3.58 3.32-3.86
Iowa 943 189 5.07 4.75-5.42 531 106 6.09 5.57-6.65 412 82 4.14 3.74-4.58
Kansas 816 163 4.97 4.63-5.33 460 92 5.94 5.39-6.52 356 71 4.12 3.69-4.59
Kentucky 1,253 251 4.84 4.57-5.12 694 139 5.78 5.35-6.24 559 112 4.00 3.67-4.36
Louisiana 1,133 227 4.37 4.11-4.63 613 123 5.19 4.78-5.63 520 104 3.69 3.37-4.03
Maine 465 93 5.13 4.65-5.65 278 56 6.58 5.80-7.44 187 37 3.88 3.31-4.53
Maryland 1,378 276 4.10 3.88-4.32 761 152 4.98 4.62-5.36 617 123 3.34 3.08-3.63
Massachusetts 1,857 371 4.60 4.39-4.82 1,040 208 5.69 5.34-6.06 817 163 3.72 3.46-3.99
Michigan 2,793 559 4.64 4.46-4.82 1,577 315 5.71 5.42-6.01 1,216 243 3.73 3.52-3.95
Minnesota 1,472 294 4.71 4.46-4.96 872 174 5.90 5.50-6.31 600 120 3.66 3.36-3.98
Mississippi 869 174 5.12 4.78-5.48 460 92 6.10 5.54-6.70 409 82 4.36 3.94-4.82
Missouri 1,596 319 4.38 4.16-4.61 887 177 5.34 4.99-5.71 709 142 3.58 3.31-3.86
Montana 312 62 4.72 4.19-5.30 178 36 5.61 4.78-6.54 134 27 3.91 3.25-4.67
Nebraska 540 108 5.05 4.62-5.51 307 61 6.08 5.41-6.82 233 47 4.16 3.63-4.76
Nevada 735 147 4.55 4.22-4.91 405 81 5.20 4.69-5.75 330 66 3.94 3.52-4.40
New Hampshire 401 80 4.83 4.35-5.35 229 46 5.82 5.06-6.67 172 34 4.00 3.40-4.69
New Jersey 2,157 431 4.12 3.94-4.30 1,200 240 5.03 4.75-5.34 957 191 3.37 3.16-3.60
New Mexico 467 93 3.79 3.45-4.16 258 52 4.44 3.90-5.03 209 42 3.23 2.79-3.71
New York 4,444 889 3.85 3.74-3.97 2,437 487 4.68 4.49-4.87 2,007 401 3.18 3.04-3.33
North Carolina 2,482 496 4.31 4.14-4.49 1,349 270 5.19 4.91-5.49 1,133 227 3.58 3.37-3.80
North Dakota 186 37 4.41 3.78-5.11 112 22 5.63 4.61-6.81 74 15 3.37 2.63-4.26
Ohio 3,263 653 4.66 4.50-4.83 1,838 368 5.69 5.43-5.97 1,425 285 3.78 3.58-3.99
Oklahoma 1,054 211 4.81 4.51-5.11 587 117 5.77 5.30-6.27 467 93 3.97 3.61-4.35
Oregon 1,214 243 4.97 4.68-5.27 694 139 5.99 5.54-6.47 520 104 4.05 3.70-4.43
Pennsylvania 3,596 719 4.46 4.31-4.61 2,023 405 5.51 5.26-5.76 1,573 315 3.60 3.41-3.79
Rhode Island 306 61 4.72 4.19-5.31 167 33 5.67 4.82-6.64 139 28 3.94 3.29-4.69
South Carolina 1,347 269 4.59 4.34-4.85 745 149 5.55 5.15-5.98 602 120 3.80 3.49-4.13
South Dakota 271 54 5.31 4.67-6.00 156 31 6.39 5.40-7.51 115 23 4.49 3.67-5.44
Tennessee 1,855 371 4.84 4.62-5.07 1,069 214 6.07 5.70-6.46 786 157 3.78 3.51-4.06
Texas 5,676 1,135 4.21 4.10-4.32 3,127 625 4.98 4.80-5.16 2,549 510 3.55 3.42-3.70
Utah 612 122 4.70 4.33-5.10 375 75 6.06 5.45-6.72 237 47 3.46 3.03-3.94
Vermont 228 46 5.76 5.01-6.61 123 25 6.71 5.53-8.08 105 21 5.00 4.05-6.14
Virginia 1,960 392 4.17 3.98-4.36 1,052 210 4.91 4.61-5.23 908 182 3.56 3.33-3.80
Washington 2,010 402 4.96 4.74-5.18 1,155 231 5.97 5.62-6.34 855 171 4.04 3.76-4.33
West Virginia 566 113 4.70 4.31-5.12 315 63 5.55 4.93-6.23 251 50 3.94 3.45-4.49
Wisconsin 1,694 339 4.93 4.69-5.18 963 193 5.93 5.55-6.33 731 146 4.01 3.72-4.33
Wyoming 180 36 5.56 4.75-6.47 93 19 5.99 4.79-7.39 87 17 5.21 4.13-6.48
United States 81,246 16,249 4.42 4.39-4.45 45,528 9,106 5.36 5.31-5.42 35,718 7,144 3.61 3.57-3.65

a. Annual average deaths are calculated by dividing the five- year total by five.

b. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

c. Estimated by CBTRUS using Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Mortality - All COD, Aggregated With State, Total U.S. (1990-2017) <Katrina/Rita Population Adjustment>, National Cancer Institute, DCCPS, Surveillance Research Program, released December 2019. Underlying mortality data provided by NCHS (www.cdc.gov/nchs).

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: NCHS, National Center for Health Statistics; CI, confidence interval.

Fig. 21.

Fig. 21

Average Annual Age-Adjusted Mortality Ratesa for Malignant Primary Brain and Other CNS Tumors by Central Cancer Registry, CBTRUS Statistical Report: NVSS, 2013-2017

  • The aggregate total number of observed deaths was 81,246, for an AAAMR rate of 4.42 per 100,000 population.

  • There was considerable variation by individual state, which ranged from a low of 2.28 deaths per 100,000 population to a high of 5.76 deaths per 100,000 population. Rates may vary by state for multiple reasons, including demographic variation and procedures for deciding primary cause of death on a death certificate.

  • Males had a higher mortality rate for malignant brain and other CNS tumors than females in the US population, with 5.36 per 100,000 population as compared to 3.61 per 100,000 population.

  • Mortality rates for malignant brain and other CNS tumors were higher in rural areas (4.7 per 100,000) as compared to urban areas (4.37 per 100,000).

  • There was considerable variation by state, where mortality rates in urban areas ranged from 2.28 per 100,000 population to 6.22 per 100,000 population, and mortality rates in rural areas ranged from 3.43 per 100,000 population to 5.79 per 100,000 population.

Estimated Incidence-Based Mortality Rates for Malignant Brain and Other CNS Tumors by Histology

Average annual age-adjusted incidence-based mortality rates for malignant primary brain and other CNS tumors by histology and behavior in the US during 2008-2017 in the SEER 9 registries are presented in Table 20. Please see the methods section for details on how these rates were calculated.

Table 20.

Ten-Year Total, Percentage of Total Deaths, and Average Annual Age-Adjusted Incidence-Based Mortality Ratesa for Brain and Other Central Nervous System Cancer Overall and by Histology, United States, CBTRUS Statistical Report: SEER, 2008-2017b

Histology Total deaths (2008-2017) % of deaths due to Brain and Other CNS Tumors attributable to this histology Rate (95% CI)
Tumors of Neuroepithelial Tissue 13,571 88.10% 4.2 (4.2-4.3)
Pilocytic Astrocytoma 95 0.60% 0.0 (0.0-0.0)
Diffuse Astrocytoma 940 6.10% 0.3 (0.3-0.3)
Anaplastic Astrocytoma 876 5.70% 0.3 (0.3-0.3)
Unique Astrocytoma Variants 62 0.40% 0.0 (0.0-0.0)
Glioblastoma 9,447 61.30% 2.9 (2.8-3.0)
Oligodendroglioma 462 3.00% 0.1 (0.1-0.2)
Anaplastic Oligodendroglioma 192 1.20% 0.1 (0.0-0.1)
Oligoastrocytic Tumors 319 2.10% 0.1 (0.1-0.1)
Ependymal Tumors 138 0.90% 0.0 (0.0-0.0)
Glioma Malignant, NOS 646 4.20% 0.2 (0.2-0.2)
Choroid Plexus Tumors -- -- --
Other Neuroepithelial Tumors -- -- --
Neuronal and Mixed Neuronal Glial Tumors 54 0.40% 0.0 (0.0-0.0)
Tumors of the Pineal Region 21 0.10% 0.0 (0.0-0.0)
Embryonal Tumors 297 1.90% 0.1 (0.1-0.1)
Tumors of Cranial and Spinal Nerves -- -- --
Nerve Sheath Tumors -- -- --
Other tumors of cranial and spinal nerves -- -- --
Tumors of Meninges 215 1.40% 0.1 (0.1-0.1)
Meningioma 139 0.90% 0.0 (0.0-0.0)
Mesenchymal tumors 51 0.30% 0.0 (0.0-0.0)
Primary melanocytic lesions -- -- --
Other neoplasms related to the meninges -- -- --
Lymphomas and Hematopoietic Neoplasms 897 5.80% 0.3 (0.3-0.3)
Lymphoma 884 5.70% 0.3 (0.3-0.3)
Other hematopoietic neoplasms -- -- --
Germ Cell Tumors and Cysts 42 0.30% 0.0 (0.0-0.0)
Germ cell tumors, cysts and heterotopias 42 0.30% 0.0 (0.0-0.0)
Tumors of Sellar Region -- -- --
Tumors of the Pituitary -- -- --
Craniopharyngioma -- -- --
Unclassified Tumors 651 4.20% 0.2 (0.2-0.2)
Hemangioma -- -- --
Neoplasm Unspecified 642 4.20% 0.2 (0.2-0.2)
All Other -- -- --
TOTAL 15,408 -- 4.8 (4.7-4.9)

a. Rates are per 100,000 and are age-adjusted to the 2000 US standard population.

b. Estimated by CBTRUS using Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Incidence - SEER Research Data, 9 Registries, Nov 2019 Sub (1975-2017) - Linked To County Attributes - Time Dependent (1990-2017) Income/Rurality, 1969-2017 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, released April 2020, based on the November 2019 submission.

- Counts and rates are not presented when fewer than 20 cases were reported for the specific category. The suppressed cases are included in the counts and rates for totals.

Abbreviations: NCHS, National Center for Health Statistics; CI, confidence interval.

  • The largest contributor to brain tumor deaths were tumors of neuroepithelial tissue (mortality rate of 4.87 per 100,000 population, 49% of total deaths).

  • Tumors of the meninges represented 32.2% of all deaths due to brain and other CNS tumors (mortality rate of 4.87 per 100,000 population, 32.2% of total deaths).

Overall Survival and Relative Survival

Overall Survival Rates for Primary Malignant Brain and Other CNS Tumors by Histology

Estimates of median survival in months by histology and age group for all individuals diagnosed with primary malignant brain and other CNS tumors regardless of whether individuals received any treatment for their tumor are presented in Table 21. Survival curves for the most common histologies are presented by age-group in Fig. 22A.

Table 21.

Sixteen-Year Total Deaths, and Median Survival in Months with 95% Confidence Intervals for Primary Malignant Brain and Other CNS Tumor Histologies, CBTRUS Statistical Report: NPCR, 2001-2016

Histology N Deaths Median survival (95% CI)
Pilocytic Astrocytoma 12,068 822 ** (** -**)
Diffuse Astrocytoma 18,420 9,662 36 (34-38)
Anaplastic Astrocytoma 13,643 9,509 18 (17-18)
Unique Astrocytoma Variants 1,564 637 44 (36-54)
Glioblastoma 113,412 103,642 8 (8-9)
Oligodendroglioma 9,062 2,362 119 (115-124)
Anaplastic Oligodendroglioma 3,943 1,723 60 (57-64)
Oligoastrocytic Tumors 6,087 2,618 71 (68-76)
Ependymal Tumors 9,344 1,831 ** (155-**)
Glioma Malignant, NOS 16,323 7,403 37 (34-42)
Choroid Plexus Tumors 310 114 77 (57-107)
Other Neuroepithelial Tumors 151 46 88 (52-**)
Neuronal and Mixed Neuronal Glial Tumors 1,907 573 93 (85-109)
Tumors of the Pineal Region 888 295 75 (68-102)
Embryonal Tumors 8,422 3,201 66 (61-73)
Nerve Sheath Tumors 467 174 95 (68-135)
Meningioma 3,807 1,771 53 (48-58)
Mesenchymal Tumors 845 337 72 (56-85)
Primary Melanocytic Lesions 140 105 11 (7-17)
Other Neoplasms Related to the Meninges 579 190 83 (70-99)
Lymphoma 15,057 10,109 13 (13-14)
Other Hemopoietic Neoplasms 392 138 84 (49-140)
Germ Cell Tumors, Cysts and Heterotopias 2,442 327 ** (** -**)
Tumors of the Pituitary 343 102 139 (113-**)
All Other 72 23 133 (35-**)
Neoplasm Unspecified 8,596 7,083 2 (2-2)

** cannot be calculated

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; CI, confidence interval; NOS, not otherwise specified

Fig. 22.

Fig. 22

A) Kaplan-Meier Survival Curves for the Five Most Common Histologies within Age Groups (Age 0-14, 15-39 and 40+) And B) Hazard Ratios And 95% Confidence Intervals for Sex, Age, Race, and Ethnicity for the Five Most Common Histologies Overall

  • Median survival was lowest for glioblastoma (8 months) and highest for malignant tumors of the pituitary (139 months, or approximately 11.5 years).

  • Median survival was not able to be estimated for pilocytic astrocytoma, ependymal tumors, or germ cell tumors as >50% of individuals remained alive during the 15 year follow up period.

  • Many other published survival estimates (including many of those previously published by CBTRUS) incorporate treatment patterns which may explain differences between these population-level estimates and other published estimates.

Demographic factors such as age at diagnosis, sex, race, and ethnicity are known to have a significant effect on survival time after diagnosis in primary brain and other CNS tumors. Hazard ratios for the effect of age groups, sex, race, and ethnicity are presented in Table 22 for all individuals regardless of whether they received any treatment for their tumor. Hazard ratio estimates for demographic factors in the five most common histologies are presented by histology in Fig. 22B.

Table 22.

Hazards Ratios† And 95% Confidence Intervals for Age Group, Sex, Race, and Ethnicity for Primary Malignant Brain and Other CNS Tumor Histologies, CBTRUS Statistical Report: NPCR, 2001-2016

Histology N Deaths Age Groups Sex Race Ethnicity (Compared to non-Hispanic)
AYAb (15-39) Years Adults (40+) Years Female Black AIAN API Hispanic
HR (95% CI) P-value HR (95% CI) P-value HR (95% CI) P-value HR (95% CI) P-value HR (95% CI) P-value HR (95% CI) P-value HR (95% CI) P-value
Pilocytic Astrocytoma 9,864 691 1.99 (1.64-2.41) <0.0001 8.38 (6.97-10.06) <0.0001 0.90 (0.78-1.05) 0.1728 1.36 (1.10-1.69) 0.0051 1.64 (0.82-3.30) 0.1639 0.47 (0.24-0.91) 0.0251 0.93 (0.73-1.18) 0.5476
Diffuse Astrocytoma 15,177 7,984 1.51 (1.33-1.72) <0.0001 5.33 (4.73-6.00) <0.0001 0.98 (0.94-1.02) 0.3615 1.08 (0.99-1.17) 0.0772 0.95 (0.74-1.22) 0.6898 0.86 (0.74-1.00) 0.0537 0.82 (0.74-0.89) <0.0001
Anaplastic Astrocytoma 11,414 7,929 0.34 (0.31-0.39) <0.0001 1.07 (0.97-1.19) 0.1800 0.99 (0.95-1.04) 0.6701 1.04 (0.95-1.13) 0.3694 1.01 (0.77-1.32) 0.9357 0.80 (0.69-0.93) 0.0041 0.78 (0.72-0.86) <0.0001
Unique Astrocytoma Variants 1,269 511 1.30 (0.93-1.82) 0.1211 5.25 (3.88-7.10) <0.0001 0.94 (0.79-1.13) 0.5260 0.72 (0.52-0.99) 0.0401 1.75 (0.86-3.54) 0.1203 0.88 (0.51-1.53) 0.6536 0.60 (0.43-0.85) 0.0034
Glioblastoma 92,191 84,294 0.72 (0.66-0.78) <0.0001 1.57 (1.46-1.70) <0.0001 1.00 (0.99-1.02) 0.6790 0.97 (0.94-1.00) 0.0407 1.02 (0.92-1.13) 0.7723 0.83 (0.79-0.88) <0.0001 0.89 (0.86-0.92) <0.0001
Oligodendroglioma 7,632 1,989 2.57 (1.54-4.30) 0.0003 5.62 (3.38-9.36) <0.0001 0.86 (0.79-0.94) 0.0012 1.38 (1.16-1.64) 0.0003 0.97 (0.54-1.76) 0.9327 0.75 (0.55-1.01) 0.0556 0.64 (0.54-0.78) <0.0001
Anaplastic Oligodendroglioma 3,320 1,474 0.36 (0.20-0.62) 0.0003 0.69 (0.40-1.20) 0.1938 0.99 (0.89-1.10) 0.8601 1.33 (1.08-1.64) 0.0065 0.46 (0.17-1.24) 0.1245 0.74 (0.55-0.99) 0.0423 0.79 (0.66-0.95) 0.0135
Oligoastrocytic Tumors 5,276 2,295 1.59 (1.08-2.34) 0.0181 3.48 (2.38-5.10) <0.0001 0.94 (0.87-1.02) 0.1592 1.21 (1.02-1.42) 0.0243 1.25 (0.81-1.92) 0.3183 0.79 (0.61-1.04) 0.0939 0.78 (0.67-0.91) 0.0016
Ependymal Tumors 7,675 1,505 0.49 (0.42-0.57) <0.0001 0.89 (0.79-1.01) 0.0610 0.75 (0.67-0.83) <0.0001 1.29 (1.10-1.50) 0.0017 1.14 (0.72-1.82) 0.5766 0.79 (0.59-1.07) 0.1341 0.88 (0.75-1.02) 0.0956
Glioma Malignant, NOS 12,440 5,388 0.72 (0.65-0.79) <0.0001 2.81 (2.64-3.00) <0.0001 0.95 (0.90-1.00) 0.0408 1.11 (1.02-1.20) 0.0156 1.05 (0.77-1.42) 0.7671 1.01 (0.86-1.19) 0.8665 1.03 (0.95-1.13) 0.4658
Choroid Plexus Tumors 240 92 0.54 (0.26-1.11) 0.0962 1.20 (0.71-2.05) 0.4942 0.90 (0.59-1.37) 0.6170 1.04 (0.56-1.94) 0.9013 2.40 (0.71-8.09) 0.1597 4.93 (2.19-11.10) 0.0001 0.43 (0.23-0.83) 0.0124
Other Neuroepithelial Tumors 121 40 1.56 (0.42-5.82) 0.5048 7.49 (2.33-24.09) 0.0007 0.48 (0.24-0.94) 0.0317 0.76 (0.22-2.61) 0.6608 ** ** ** ** 0.69 (0.23-2.03) 0.4994
Neuronal and Mixed Neuronal Glial Tumors 1,563 475 1.24 (0.82-1.89) 0.3086 1.49 (1.01-2.20) 0.0423 0.85 (0.70-1.02) 0.0782 1.30 (0.98-1.72) 0.0670 3.30 (1.62-6.71) 0.0010 0.90 (0.58-1.38) 0.6145 0.94 (0.70-1.27) 0.6923
Tumors of the Pineal Region 740 246 0.58 (0.42-0.80) 0.0008 1.00 (0.73-1.36) 0.9817 0.69 (0.54-0.89) 0.0049 0.99 (0.73-1.34) 0.9373 1.08 (0.39-2.94) 0.8864 0.38 (0.14-1.03) 0.0580 1.08 (0.75-1.56) 0.6697
Embryonal Tumors 6,834 2,605 0.80 (0.73-0.88) <0.0001 1.66 (1.48-1.86) <0.0001 1.01 (0.94-1.09) 0.7680 1.27 (1.13-1.43) <0.0001 1.13 (0.76-1.68) 0.5392 0.95 (0.77-1.18) 0.6344 0.84 (0.76-0.93) 0.0012
Nerve Sheath Tumors 339 127 0.71 (0.32-1.56) 0.3922 0.86 (0.41-1.77) 0.6784 0.71 (0.50-1.02) 0.0631 1.31 (0.80-2.15) 0.2891 ** ** 0.09 (0.01-0.65) 0.0167 1.23 (0.66-2.26) 0.5162
Meningioma 2,736 1,188 0.84 (0.43-1.66) 0.6184 2.11 (1.13-3.93) 0.0192 0.73 (0.65-0.81) <0.0001 0.96 (0.83-1.12) 0.6068 0.57 (0.21-1.52) 0.2619 0.74 (0.55-1.01) 0.0544 0.70 (0.56-0.89) 0.0034
Mesenchymal Tumors 704 278 0.70 (0.45-1.09) 0.1161 0.98 (0.67-1.43) 0.9064 0.98 (0.77-1.24) 0.8657 1.10 (0.75-1.62) 0.6282 1.31 (0.48-3.55) 0.5956 0.56 (0.31-1.00) 0.0496 0.93 (0.63-1.36) 0.7010
Primary Melanocytic Lesions 100 76 0.42 (0.17-1.02) 0.0548 0.63 (0.30-1.31) 0.2140 0.83 (0.50-1.37) 0.4595 1.91 (0.66-5.49) 0.2321 ** ** 1.19 (0.42-3.39) 0.7463 2.13 (0.89-5.11) 0.0897
Other Neoplasms Related to the Meninges 443 144 1.34 (0.46-3.93) 0.5880 3.09 (1.11-8.61) 0.0305 0.83 (0.59-1.16) 0.2743 0.99 (0.56-1.78) 0.9854 3.12 (0.98-9.90) 0.0535 0.63 (0.25-1.58) 0.3281 1.01 (0.59-1.73) 0.9620
Lymphoma 12,068 7,997 2.06 (1.21-3.50) 0.0079 3.74 (2.21-6.32) <0.0001 0.93 (0.89-0.97) 0.0018 1.21 (1.12-1.31) <0.0001 1.32 (1.03-1.70) 0.0276 0.86 (0.77-0.95) 0.0049 0.87 (0.80-0.94) 0.0006
Other Hemopoietic Neoplasms 316 112 3.14 (0.87-11.28) 0.0799 9.46 (2.98-30.04) 0.0001 0.85 (0.58-1.24) 0.3943 0.82 (0.48-1.39) 0.4568 0.98 (0.24-4.04) 0.9774 0.35 (0.09-1.45) 0.1490 1.09 (0.64-1.86) 0.7455
Germ Cell Tumors, Cysts and Heterotopias 2,027 269 0.98 (0.75-1.27) 0.8531 3.66 (2.35-5.70) <0.0001 1.43 (1.09-1.89) 0.0110 0.85 (0.55-1.31) 0.4671 1.94 (0.62-6.11) 0.2561 0.60 (0.38-0.96) 0.0331 0.84 (0.61-1.16) 0.2863
Tumors of the Pituitary 261 77 ** ** ** ** 0.58 (0.36-0.93) 0.0252 1.58 (0.94-2.65) 0.0866 1.16 (0.16-8.71) 0.8827 0.37 (0.05-2.73) 0.3320 0.76 (0.30-1.91) 0.5587
Neoplasm Unspecified 5,013 4,081 0.71 (0.56-0.90) 0.0040 2.32 (1.93-2.78) <0.0001 0.98 (0.92-1.04) 0.4526 0.73 (0.66-0.81) <0.0001 0.95 (0.67-1.34) 0.7667 0.90 (0.73-1.11) 0.3268 0.89 (0.78-1.00) 0.0541

a.Children as defined by the National Cancer Institute, see: http://www.cancer.gov/researchandfunding/snapshots/pediatric.

b.Adolescents and Young Adults (AYA), as defined by the National Cancer Institute, see: http://www.cancer.gov/cancertopics/aya.

Reference Categories are Children (<14 years) for Age group, Male for Sex, White for Race, and Non-Hispanic for Ethnicity.

** Cannot be calculated

Abbreviations: AYA, Adolescents and Young Adults; CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; CI, confidence interval; NOS, not otherwise specified

  • AYA had better overall survival as compared to children 0-14 years old in approximately half of the histologies evaluated, while adults 40+ years old had poorer survival.

  • Older adults (40+ years old) had poorer survival than Children 0-14 years old in nearly every histology.

  • Females generally had better survival outcomes as compared to males with the exception of glioblastoma, embryonal tumors, and germ cell tumors.

  • Black individuals had poorer survival outcomes as compared to white individuals with the exception of glioblastoma.

  • AIAN individuals had poorer survival as compared to white individuals in many histologies, though the small size of this population meant that many of these associations were non-significant.

  • Being an API was associated with improved survival in many histologies as compared to Whites with the exception of choroid plexus tumors.

  • Hispanic ethnicity was associated with improved survival in most histologies.

  • Many other published survival estimates (including many of those previously published by CBTRUS 62,63 ) incorporate treatment patterns which may explain differences between these population-level estimates and other published estimates.

When interpreting these results, it is important to remember that these models do not incorporate important factors that affect survival such as treatment patterns, health insurance, or type of facility at which an individual received treatment, all of which may be associated with these demographic factors as well as overall survival.

Relative Survival Rates for Brain and Other CNS Tumors by Site and Behavior

Relative survival estimates by site and behavior are presented in Supplementary Table 12

  • The highest five-year survival was for tumors occurring in the cranial nerves (99.3%).

  • The lowest five-year survival was for tumors of the parietal lobe (27.7%).

Relative Survival Rates for Brain and Other CNS Tumors by Histology, Behavior and Age Groups

Relative survival estimates for brain and other CNS tumors by histology, behavior, and age at diagnosis are presented in Table 23 and Supplementary Table 13.

Table 23.

One-, Five-, and Ten-Year Relative Survival Ratesa,b with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors Overall and by NCI Age Groups, CBTRUS Statistical Report: NPCR, 2004-2016c

Histology All ages 0-14 years old 15-39 years old 40+ years old
N 1 year 5 year 10 year N 1 year 5 year 10 year N 1 year 5 year 10 year N 1 year 5 year 10 year
Pilocytic Astrocytoma 12,067 98.0 (97.7-98.2) 94.5 (94.0-95.0) 93.0 (92.4-93.6) 7,178 98.9 (98.6-99.1) 97.1 (96.6-97.5) 96.0 (95.3-96.5) 3,654 98.4 (97.9-98.8) 94.8 (93.9-95.5) 92.9 (91.7-93.9) 1,235 91.3 (89.4-92.8) 78.6 (75.7-81.3) 76.1 (72.4-79.4)
Diffuse Astrocytoma 18,450 76.3 (75.6-76.9) 53.0 (52.2-53.8) 43.1 (42.1-44.0) 1,947 92.9 (91.6-93.9) 83.8 (82.0-85.4) 81.2 (79.1-83.1) 5,801 95.7 (95.1-96.2) 78.1 (76.8-79.2) 61.9 (60.1-63.6) 10,702 62.7 (61.7-63.6) 33.5 (32.5-34.6) 25.6 (24.5-26.7)
Anaplastic Astrocytoma 13,656 66.4 (65.6-67.2) 31.1 (30.2-32.0) 22.7 (21.7-23.7) 621 66.2 (62.3-69.8) 24.8 (21.2-28.6) 19.6 (15.9-23.7) 3,577 91.9 (90.9-92.7) 61.8 (59.9-63.7) 45.7 (43.2-48.1) 9,458 56.9 (55.8-57.9) 20.0 (19.1-20.9) 14.3 (13.3-15.3)
Unique Astrocytoma Variants 2,506 87.4 (86.0-88.7) 74.0 (72.1-75.9) 69.9 (67.6-72.2) 908 97.3 (96.0-98.2) 92.0 (89.8-93.8) 88.8 (85.6-91.4) 897 95.6 (94.0-96.8) 85.2 (82.4-87.6) 80.6 (77.1-83.7) 701 63.7 (59.8-67.3) 34.7 (30.6-38.8) 29.5 (24.8-34.4)
Malignant 1,566 81.4 (79.3-83.3) 60.7 (57.9-63.3) 55.9 (52.7-59.0) 331 95.0 (91.9-96.9) 81.7 (76.5-85.9) 75.6 (68.4-81.4) 606 95.1 (93.0-96.6) 80.7 (76.8-84.0) 76.0 (71.3-80.0) 629 60.5 (56.4-64.4) 29.1 (25.0-33.4) 24.8 (20.2-29.7)
Non-Malignant 940 97.4 (96.1-98.3) 95.3 (93.5-96.6) 92.1 (89.1-94.3) 577 98.6 (97.2-99.3) 97.6 (95.9-98.7) 96.0 (92.6-97.9) 291 96.6 (93.6-98.2) 94.1 (90.2-96.4) 89.6 (83.4-93.6) 72 90.7 (80.4-95.7) 80.6 (67.4-88.9) 68.7 (48.3-82.4)
Glioblastoma 113,422 42.8 (42.5-43.1) 7.2 (7.0-7.4) 4.7 (4.6-4.9) 1,040 57.5 (54.4-60.5) 19.6 (17.0-22.3) 16.3 (13.8-19.1) 5,809 76.3 (75.1-77.4) 26.8 (25.5-28.1) 18.8 (17.5-20.2) 106,573 40.8 (40.5-41.1) 5.9 (5.8-6.1) 3.8 (3.6-4.0)
Oligodendroglioma 9,068 94.9 (94.4-95.3) 83.4 (82.5-84.3) 69.6 (68.2-71.0) 294 97.6 (94.9-98.8) 94.1 (90.5-96.4) 91.9 (87.5-94.8) 3,656 98.6 (98.1-98.9) 91.7 (90.5-92.6) 76.4 (74.2-78.4) 5,118 92.0 (91.2-92.8) 76.8 (75.4-78.1) 63.2 (61.2-65.2)
Anaplastic Oligodendroglioma 3,946 87.7 (86.6-88.7) 64.0 (62.2-65.7) 49.7 (47.4-52.0) -- -- -- -- 1,131 95.8 (94.4-96.8) 78.4 (75.5-81.0) 60.8 (56.6-64.6) 2,779 84.4 (83.0-85.8) 58.0 (55.9-60.1) 45.0 (42.2-47.8)
Oligoastrocytic Tumors 6,092 89.6 (88.8-90.4) 67.6 (66.3-68.9) 52.3 (50.6-54.0) 162 91.3 (85.7-94.8) 80.5 (73.3-86.0) 78.6 (71.0-84.5) 2,687 97.5 (96.9-98.1) 81.6 (79.9-83.1) 61.1 (58.5-63.7) 3,243 82.9 (81.6-84.2) 55.2 (53.3-57.0) 43.5 (41.1-45.8)
Ependymal Tumors 15,690 96.0 (95.6-96.3) 90.6 (89.9-91.1) 86.3 (85.4-87.2) 2,239 95.7 (94.8-96.5) 80.4 (78.4-82.1) 71.5 (68.9-74.0) 4,643 98.2 (97.8-98.6) 94.9 (94.1-95.6) 91.4 (90.1-92.5) 8,808 94.9 (94.3-95.4) 90.9 (90.0-91.8) 87.5 (86.1-88.9)
Malignant 9,170 94.8 (94.3-95.3) 86.5 (85.7-87.4) 81.0 (79.7-82.2) 1,987 95.3 (94.3-96.2) 78.3 (76.2-80.3) 68.5 (65.7-71.2) 2,567 97.2 (96.5-97.8) 91.9 (90.7-93.1) 87.1 (85.1-88.7) 4,616 93.3 (92.4-94.0) 87.1 (85.8-88.3) 83.0 (81.0-84.8)
Non-Malignant 6,520 97.7 (97.2-98.1) 96.4 (95.6-97.1) 94.4 (92.9-95.6) 252 98.8 (96.2-99.6) 96.8 (93.1-98.5) 96.1 (92.0-98.1) 2,076 99.5 (99.0-99.7) 98.6 (97.7-99.2) 97.0 (95.4-98.1) 4,192 96.7 (96.0-97.3) 95.3 (94.0-96.3) 92.9 (90.5-94.7)
Glioma Malignant, NOS 16,380 71.9 (71.2-72.6) 57.5 (56.6-58.3) 52.8 (51.8-53.8) 5,711 82.9 (81.9-83.9) 70.5 (69.2-71.8) 69.4 (68.0-70.7) 3,378 92.6 (91.7-93.5) 79.4 (77.8-80.9) 71.7 (69.5-73.8) 7,291 53.2 (52.0-54.4) 36.5 (35.2-37.7) 30.0 (28.5-31.6)
Choroid Plexus Tumors 1,986 93.8 (92.6-94.8) 89.4 (87.7-90.9) 86.1 (83.6-88.1) 868 95.2 (93.5-96.5) 89.9 (87.4-91.9) 87.5 (84.5-90.0) 524 97.9 (96.2-98.9) 95.8 (93.3-97.4) 91.8 (87.6-94.7) 594 88.1 (84.9-90.6) 83.0 (78.7-86.6) 78.6 (71.9-83.8)
Malignant 308 86.0 (81.4-89.5) 68.8 (62.6-74.2) 57.9 (50.4-64.8) 229 86.6 (81.4-90.5) 68.6 (61.3-74.9) 61.1 (52.5-68.6) -- -- -- -- -- -- -- --
Non-Malignant 1,678 95.2 (94.0-96.2) 93.2 (91.5-94.5) 91.1 (88.6-93.1) 639 98.3 (96.8-99.1) 97.2 (95.3-98.3) 96.4 (93.8-97.9) -- -- -- -- -- -- -- --
Other Neuroepithelial Tumors 240 84.3 (78.8-88.5) 72.5 (65.5-78.4) 66.5 (57.7-73.8) 64 96.7 (87.1-99.2) 88.4 (75.6-94.7) 88.4 (75.6-94.7) 78 94.7 (86.2-98.0) 87.8 (76.5-93.9) 83.1 (69.8-90.9) 98 68.4 (57.7-76.8) 50.3 (38.5-61.0) 39.9 (26.1-53.3)
Malignant 151 84.4 (77.2-89.5) 70.7 (61.5-78.1) 62.9 (51.2-72.5) -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 89 84.2 (74.3-90.5) 75.6 (63.7-84.1) 71.8 (57.2-82.1) -- -- -- -- -- -- -- -- -- -- -- --
Neuronal and Mixed Neuronal Glial Tumors 10,632 96.8 (96.4-97.1) 92.0 (91.3-92.6) 89.0 (88.0-89.9) 2,751 98.6 (98.0-99.0) 95.8 (94.8-96.5) 94.7 (93.5-95.7) 4,370 98.2 (97.8-98.6) 95.2 (94.4-95.9) 91.9 (90.6-93.0) 3,511 93.5 (92.5-94.3) 84.8 (83.2-86.3) 80.6 (78.3-82.6)
Malignant 1,930 91.5 (90.0-92.7) 76.9 (74.5-79.1) 69.9 (66.6-73.0) 167 88.2 (82.0-92.3) 77.0 (69.1-83.1) 75.9 (67.8-82.2) 465 93.9 (91.2-95.8) 76.3 (71.7-80.3) 66.5 (60.2-72.1) 1,298 91.0 (89.2-92.6) 77.1 (74.0-79.9) 69.9 (65.6-73.8)
Non-Malignant 8,702 97.9 (97.6-98.2) 95.3 (94.7-95.8) 93.2 (92.3-94.1) 2,584 99.2 (98.8-99.5) 97.0 (96.1-97.7) 95.9 (94.7-96.8) 3,905 98.8 (98.3-99.1) 97.5 (96.8-98.0) 95.0 (93.8-96.0) 2,213 94.9 (93.8-95.8) 89.3 (87.4-90.9) 86.7 (84.0-89.0)
Tumors of the Pineal Region 1,659 92.3 (90.8-93.6) 79.3 (76.8-81.5) 72.9 (69.5-75.9) 349 90.1 (86.4-92.9) 69.2 (63.4-74.3) 62.6 (56.0-68.5) 629 95.3 (93.2-96.7) 85.3 (81.8-88.2) 80.3 (75.5-84.3) 681 90.7 (88.0-92.8) 79.0 (74.7-82.6) 71.3 (64.9-76.7)
Malignant 893 89.8 (87.6-91.7) 68.8 (65.1-72.3) 59.5 (54.7-63.9) 295 88.7 (84.4-91.9) 63.6 (56.9-69.4) 56.1 (48.7-62.9) 318 92.8 (89.2-95.2) 73.6 (67.3-78.8) 64.6 (56.4-71.6) 280 87.6 (82.8-91.1) 69.1 (61.9-75.2) 56.5 (47.1-64.9)
Non-Malignant 766 95.2 (93.2-96.6) 91.4 (88.3-93.7) 88.4 (83.5-91.9) 54 98.1 (86.7-99.7) 98.1 (86.7-99.7) 94.8 (78.8-98.8) 311 97.7 (95.2-98.9) 96.8 (93.5-98.4) 95.5 (90.9-97.8) 401 92.8 (89.4-95.1) 85.9 (80.3-90.0) 81.2 (72.1-87.5)
Embryonal Tumors 8,716 83.4 (82.6-84.2) 64.6 (63.5-65.7) 58.1 (56.8-59.4) 5,722 82.0 (80.9-83.0) 63.9 (62.5-65.2) 59.2 (57.6-60.6) 2,114 91.5 (90.1-92.6) 72.0 (69.8-74.0) 61.8 (59.1-64.4) 880 73.5 (70.3-76.3) 51.1 (47.3-54.7) 42.8 (38.3-47.1)
Malignant 8,452 82.9 (82.1-83.7) 63.5 (62.4-64.7) 57.0 (55.7-58.3) 5,667 81.8 (80.7-82.8) 63.6 (62.2-64.9) 58.7 (57.2-60.3) 2,021 91.1 (89.7-92.2) 70.7 (68.4-72.8) 60.3 (57.4-62.9) 764 70.0 (66.5-73.2) 44.6 (40.6-48.5) 35.7 (31.1-40.4)
Non-Malignant 264 98.1 (95.0-99.3) 97.3 (92.1-99.1) 93.3 (85.3-97.0) 55 98.2 (87.3-99.8) 98.2 (87.3-99.8) 98.2 (87.3-99.8) 93 100.0 (**-**) 100.0 (**-**) 97.7 (65.7-99.9) 116 96.5 (89.6-98.9) 94.2 (83.1-98.1) 87.5 (72.4-94.7)
Nerve Sheath Tumors 76,644 99.2 (99.1-99.3) 99.2 (99.1-99.3) 99.2 (99.1-99.3) 2,095 99.7 (99.3-99.9) 98.7 (98.0-99.1) 98.1 (97.2-98.7) 11,798 99.3 (99.1-99.4) 98.4 (98.1-98.7) 97.4 (96.9-97.9) 62,751 99.2 (99.0-99.3) 99.2 (99.0-99.3) 99.2 (99.0-99.3)
Malignant 472 82.8 (78.9-86.1) 70.1 (65.0-74.6) 67.8 (61.2-73.6) -- -- -- -- 121 81.1 (72.7-87.2) 66.9 (57.2-74.9) 64.6 (54.6-73.0) 318 83.0 (78.0-86.9) 71.0 (64.4-76.6) 68.3 (58.9-76.0)
Non-Malignant 76,172 99.3 (99.2-99.4) 99.3 (99.2-99.4) 99.3 (99.2-99.4) -- -- -- -- 11,677 99.5 (99.3-99.6) 98.7 (98.4-99.0) 97.8 (97.2-98.2) 62,433 99.2 (99.1-99.4) 99.2 (99.1-99.4) 99.2 (99.1-99.4)
Other Tumors of Cranial and Spinal Nerves 58 95.7 (82.6-99.0) 90.4 (73.8-96.7) 82.4 (57.7-93.4) -- -- -- -- -- -- -- -- -- -- -- --
Meningioma 328,639 93.0 (92.9-93.2) 87.9 (87.7-88.1) 83.4 (83.1-83.8) 619 97.6 (95.9-98.5) 94.2 (91.8-95.9) 89.1 (85.0-92.1) 20,793 98.7 (98.5-98.9) 96.8 (96.5-97.1) 94.5 (94.0-95.0) 307,227 92.6 (92.5-92.8) 87.2 (87.0-87.4) 82.6 (82.2-82.9)
Malignant 3,820 83.9 (82.5-85.1) 66.6 (64.6-68.5) 59.6 (57.0-62.1) 52 90.0 (77.4-95.8) 77.6 (61.9-87.4) 70.3 (52.5-82.4) 303 93.0 (89.4-95.4) 81.3 (75.9-85.6) 73.6 (66.3-79.5) 3,465 82.9 (81.5-84.3) 65.0 (62.9-67.1) 58.0 (55.4-60.6)
Non-Malignant 324,819 93.2 (93.0-93.3) 88.1 (87.9-88.3) 83.7 (83.4-84.1) 567 98.2 (96.7-99.1) 95.6 (93.3-97.1) 90.7 (86.5-93.7) 20,490 98.8 (98.6-98.9) 97.1 (96.8-97.3) 94.9 (94.4-95.3) 303,762 92.8 (92.6-92.9) 87.5 (87.3-87.7) 82.9 (82.5-83.2)
Mesenchymal Tumors 3,522 94.0 (93.1-94.8) 86.3 (84.8-87.7) 79.2 (76.7-81.4) 720 96.6 (94.9-97.8) 92.3 (89.7-94.3) 91.3 (88.4-93.5) 826 96.8 (95.3-97.9) 91.9 (89.4-93.8) 86.8 (83.0-89.9) 1,976 91.9 (90.4-93.1) 81.9 (79.5-84.0) 72.3 (68.5-75.7)
Malignant 854 84.7 (82.0-87.1) 65.5 (61.5-69.1) 53.8 (48.3-59.0) 112 79.6 (70.7-86.1) 64.8 (54.2-73.5) 63.0 (52.0-72.1) 212 90.3 (85.3-93.7) 74.6 (67.3-80.6) 64.5 (54.6-72.8) 530 83.5 (79.8-86.6) 61.7 (56.4-66.6) 47.6 (40.1-54.7)
Non-Malignant 2,668 97.0 (96.1-97.6) 93.0 (91.5-94.3) 87.4 (84.7-89.7) 608 99.8 (96.4-100.0) 97.8 (95.6-98.9) 96.9 (94.3-98.3) 614 99.1 (97.8-99.6) 97.7 (95.6-98.8) 94.5 (90.4-96.9) 1,446 94.9 (93.4-96.0) 89.1 (86.5-91.2) 81.2 (76.8-84.8)
Primary Melanocytic Lesions 228 65.7 (58.9-71.6) 44.7 (37.1-52.0) 33.3 (24.1-42.7) -- -- -- -- -- -- -- -- 162 66.6 (58.4-73.5) 43.0 (33.8-51.8) 32.6 (22.0-43.6)
Malignant 141 52.7 (43.9-60.8) 32.0 (23.5-40.7) 22.4 (12.5-34.1) -- -- -- -- -- -- -- -- 99 55.9 (45.2-65.4) 32.9 (22.7-43.5) 25.8 (13.2-40.3)
Non-Malignant 87 86.8 (76.9-92.6) 64.8 (51.4-75.3) 49.1 (32.4-63.8) -- -- -- -- -- -- -- -- 63 83.3 (70.6-90.9) 57.7 (41.0-71.2) 41.4 (23.4-58.5)
Other Neoplasms Related to the Meninges 7,032 96.2 (95.6-96.6) 92.9 (92.0-93.7) 88.7 (87.1-90.1) 135 97.8 (93.2-99.3) 93.1 (86.3-96.6) 86.2 (75.2-92.5) 2,212 98.3 (97.6-98.8) 96.0 (94.9-96.9) 93.5 (91.7-94.9) 4,685 95.1 (94.3-95.8) 91.4 (90.1-92.5) 86.4 (84.1-88.3)
Malignant 610 94.4 (91.9-96.1) 78.9 (74.4-82.7) 67.9 (61.5-73.5) -- -- -- -- 179 96.6 (92.4-98.5) 86.6 (79.7-91.2) 84.7 (77.2-89.9) 401 93.2 (89.7-95.5) 74.5 (68.2-79.7) 58.3 (49.5-66.2)
Non-Malignant 6,422 96.3 (95.8-96.8) 94.2 (93.3-95.0) 90.7 (89.1-92.1) -- -- -- -- 2,033 98.4 (97.7-98.9) 96.9 (95.8-97.6) 94.2 (92.4-95.6) 4,284 95.3 (94.5-96.0) 92.9 (91.6-94.1) 89.0 (86.6-91.0)
Lymphoma 15,058 55.0 (54.2-55.8) 37.6 (36.7-38.5) 29.7 (28.6-30.8) 66 90.5 (80.0-95.6) 78.3 (65.4-86.8) 68.6 (52.1-80.4) 1,344 65.2 (62.5-67.7) 56.8 (54.0-59.6) 52.0 (48.7-55.2) 13,648 53.8 (52.9-54.7) 35.3 (34.4-36.3) 27.0 (25.8-28.2)
Other Hemopoietic Neoplasms 395 83.6 (79.3-87.1) 67.3 (61.4-72.5) 65.3 (57.6-71.9) 80 94.7 (86.4-98.0) 94.7 (86.4-98.0) 89.5 (71.2-96.5) 78 91.6 (82.2-96.2) 75.7 (61.2-85.4) 75.7 (61.2-85.4) 237 77.4 (71.2-82.5) 56.3 (48.6-63.3) 54.8 (45.5-63.2)
Germ Cell Tumors, Cysts and Heterotopias 3,702 94.8 (94.0-95.5) 90.3 (89.2-91.4) 87.6 (86.0-89.0) 1,507 94.3 (93.0-95.4) 90.6 (88.9-92.1) 88.0 (85.7-90.0) 1,676 96.1 (95.0-97.0) 90.7 (89.0-92.2) 89.3 (87.3-91.0) 519 92.3 (89.3-94.5) 88.3 (83.8-91.6) 80.6 (73.1-86.2)
Malignant 2,450 93.7 (92.6-94.6) 87.9 (86.4-89.3) 85.3 (83.4-87.0) 1,105 93.2 (91.5-94.6) 88.3 (86.1-90.2) 84.9 (82.0-87.4) 1,275 95.1 (93.7-96.2) 88.9 (86.8-90.6) 87.2 (84.7-89.2) 70 75.9 (63.5-84.6) 65.5 (51.4-76.4) 54.2 (34.6-70.2)
Non-Malignant 1,252 97.1 (95.8-98.0) 95.0 (93.0-96.5) 92.1 (88.9-94.4) 402 97.3 (95.0-98.5) 97.0 (94.6-98.3) 96.6 (93.9-98.1) 401 99.3 (97.5-99.8) 96.6 (93.6-98.2) 95.5 (91.2-97.7) 449 94.9 (91.9-96.8) 91.9 (86.9-95.0) 84.8 (76.4-90.5)
Tumors of the Pituitary 145,970 98.1 (98.0-98.2) 96.8 (96.6-97.0) 95.2 (94.8-95.5) 1,967 99.9 (99.5-100.0) 99.4 (98.8-99.7) 98.9 (98.0-99.4) 43,972 99.7 (99.6-99.7) 99.3 (99.1-99.4) 98.6 (98.4-98.8) 100,031 97.4 (97.3-97.5) 95.7 (95.4-95.9) 93.5 (93.0-94.0)
Malignant 346 91.1 (87.1-93.8) 81.9 (75.8-86.5) 78.7 (69.7-85.2) -- -- -- -- 63 100.0 (**-**) 90.0 (76.2-96.0) 90.0 (76.2-96.0) 280 88.9 (84.1-92.3) 79.8 (72.6-85.2) 75.4 (64.4-83.4)
Non-Malignant 145,624 98.2 (98.1-98.2) 96.9 (96.7-97.0) 95.2 (94.9-95.6) -- -- -- -- 43,909 99.7 (99.6-99.7) 99.3 (99.2-99.4) 98.6 (98.4-98.8) 99,751 97.4 (97.3-97.6) 95.7 (95.4-96.0) 93.5 (93.0-94.0)
Craniopharyngioma 7,204 93.1 (92.5-93.7) 86.2 (85.2-87.2) 80.5 (79.0-81.9) 1,649 98.6 (97.9-99.1) 95.4 (94.1-96.4) 92.5 (90.6-94.1) 1,743 96.1 (95.0-96.9) 91.6 (90.0-93.0) 88.2 (86.1-90.1) 3,812 89.3 (88.2-90.3) 79.5 (77.8-81.1) 70.9 (68.3-73.3)
Hemangioma 11,408 96.8 (96.4-97.2) 94.0 (93.3-94.6) 91.7 (90.4-92.9) 712 99.2 (98.1-99.7) 97.7 (96.0-98.7) 97.7 (96.0-98.7) 3,197 99.5 (99.2-99.7) 98.8 (98.1-99.2) 96.8 (95.4-97.8) 7,499 95.4 (94.8-95.9) 91.5 (90.4-92.5) 88.8 (86.7-90.6)
Neoplasm Unspecified 24,689 60.9 (60.3-61.6) 53.1 (52.3-53.8) 49.1 (48.2-50.0) 1,294 87.2 (85.2-88.9) 84.1 (81.8-86.0) 82.5 (80.0-84.8) 3,829 93.0 (92.1-93.8) 89.6 (88.5-90.6) 87.8 (86.4-89.1) 19,566 52.7 (51.9-53.4) 43.5 (42.7-44.3) 38.6 (37.5-39.7)
Malignant 8,596 31.2 (30.2-32.2) 21.7 (20.7-22.7) 19.3 (18.2-20.5) 322 63.2 (57.5-68.3) 54.4 (48.4-59.9) 51.9 (45.7-57.7) 632 78.4 (75.0-81.5) 68.2 (64.1-72.0) 65.2 (60.6-69.3) 7,642 25.8 (24.7-26.8) 16.2 (15.3-17.2) 13.9 (12.8-15.0)
Non-Malignant 16,093 76.6 (75.9-77.3) 69.7 (68.8-70.6) 64.8 (63.7-66.0) 972 95.1 (93.5-96.4) 93.9 (92.1-95.3) 92.7 (90.2-94.6) 3,197 95.9 (95.1-96.5) 93.8 (92.8-94.7) 92.3 (91.0-93.5) 11,924 69.8 (68.9-70.7) 60.9 (59.8-62.0) 54.5 (52.9-56.0)
All Other 327 83.4 (78.2-87.4) 74.3 (67.0-80.2) 67.8 (58.3-75.7) -- -- -- -- 50 93.8 (81.6-98.0) 77.2 (61.3-87.2) 77.2 (61.3-87.2) 228 79.7 (72.9-85.0) 73.5 (63.7-81.0) 63.2 (49.9-73.8)
Malignant 78 78.0 (66.2-86.0) 59.6 (45.6-71.1) 56.5 (41.2-69.2) -- -- -- -- -- -- -- -- -- -- -- --
Non-Malignant 249 85.1 (79.1-89.5) 78.9 (70.5-85.1) 71.5 (59.2-80.6) -- -- -- -- -- -- -- -- -- -- -- --
TOTALd 859,821 85.1 (85.0-85.1) 75.2 (75.1-75.3) 71.5 (71.3-71.7) 40,843 91.4 (91.1-91.7) 82.5 (82.1-82.9) 79.9 (79.5-80.4) 134,619 96.8 (96.7-96.9) 90.4 (90.2-90.6) 86.0 (85.8-86.3) 684,359 82.3 (82.2-82.4) 71.7 (71.5-71.8) 68.0 (67.8-68.2)
Malignant 248,005 61.1 (60.9-61.3) 36.0 (35.8-36.2) 30.9 (30.6-31.1) 27,371 87.7 (87.3-88.1) 75.4 (74.8-75.9) 72.2 (71.6-72.8) 39,902 91.3 (91.0-91.5) 72.5 (72.0-73.0) 61.9 (61.2-62.5) 180,732 50.3 (50.0-50.5) 21.5 (21.3-21.7) 17.2 (17.0-17.4)
Non-Malignant 611,816 94.9 (94.9-95.0) 91.7 (91.5-91.8) 88.8 (88.6-89.0) 13,472 98.8 (98.6-99.0) 97.3 (97.0-97.6) 96.0 (95.5-96.4) 94,717 99.2 (99.1-99.2) 98.2 (98.0-98.3) 96.8 (96.6-97.0) 503,627 94.0 (93.9-94.1) 90.2 (90.1-90.4) 87.0 (86.7-87.2)

a.The cohort analysis of survival rates was utilized for calculating the survival estimates presented in this table. Long-term cohort-based survival estimates reflect the survival experience of individuals diagnosed over the time period, and they may not necessarily reflect the long-term survival outlook of newly diagnosed cases.

b.Rates are an estimate of the percentage of patients alive at one, two, five, and ten years, respectively. Rates were not presented for categories with 50 or fewer cases and were suppressed for rates where fewer than 16 cases were surviving within a category.

c.Total number of cases that occurred within the NPCR registries between 2004 and 2016.

d.Total includes histologies not listed in this table.

- Rates were not presented for categories with 50 or fewer cases and were suppressed for rates where fewer than 16 cases were surviving within a category.

** Confidence interval could not be calculated

Abbreviations: CBTRUS, Central Brain Tumor Registry of the United States; NPCR, National Program of Cancer Registries; CI, confidence interval; NOS, not otherwise specified

  • There was large variation in survival estimates depending upon tumor histology; five-year survival rates were 94.5% for pilocytic astrocytoma but are 7.2% for glioblastoma.

  • Survival generally decreased with older age at diagnosis; children and young adults generally had better survival outcomes for most histologies.

  • Among predominantly non-malignant histologies, five-year survival was lowest in craniopharyngioma and meningioma, which had five-year relative survival of 86.2% and 88.1%, respectively.

  • Among predominantly non-malignant histologies, five-year survival was highest in nerve sheath tumors which had five-year relative survival of 99.3%.

  • In general, relative survival in most histologies was higher in adolescents and young adults as compared to children and adults.

Relative Survival Rates for Brain and Other CNS Tumors by Histology, Behavior, and Urban/Rural Residence

Survival estimates for primary malignant and non-malignant brain and other CNS tumors are presented by urban/rural residence and selected histologies in Supplementary Table 14. Overall, one-, five-, and ten-year survival were higher in urban areas as compared to rural areas.

Descriptive Summary of Spinal Cord Tumors

Although spinal cord tumors account for a relatively small percentage of primary brain and other CNS tumors, they can result in significant morbidity. The most common histologies found in the spinal cord, spinal meninges, and cauda equina are presented in Fig. 23 for both children (age 0-19 years, Fig. 23A) and adults (age 20+ years, Fig. 23B).

  • The predominant histology group for those age 0-19 years was ependymal tumors (19.6%) followed by tumors of meninges (17.8%).

  • Tumors of meninges (39.5%) accounted for the largest proportion of spinal cord tumors among those age 20 years and older.

  • Five-year survival after diagnosis with a tumor of the spinal cord and cauda equina was 93.6%, with a ten-year relative survival of 92.1% Supplementary Table 12.

Descriptive Summary of Meningioma, Glioblastoma, and Embryonal Tumors

The data in the CBTRUS Statistical Report 2013-2017 are synthesized to describe three of the most common histologic types: meningioma and glioblastoma for adults, and embryonal tumors for children and adolescents.

Meningioma

  • Meningioma was the most frequently reported brain and other CNS tumor, accounting for 38.3% of tumors overall (Fig. 6B).

  • Most meningiomas (80.6%) were located in the cerebral meninges, 4.2% were located in the spinal meninges, and approximately 14.5% did not have a specific meningeal site listed.

  • Non-malignant meningioma with ICD-O-3 behavior codes /0 (benign) or /1 (uncertain) accounted for 98.9% of meningiomas reported to CBTRUS (Table 5).

  • Of meningioma with documented WHO grade (81.3%, Table 11), 80.3% of meningioma were WHO grade I, 17.9% were WHO grade II, and 1.6% were WHO grade III.

  • Meningioma was most common in adults age 65 years and older (Table 7), and one of the least common in children age 0-14 years (Table 5).

  • Incidence of meningioma increased with age, with a dramatic increase after age 65 years. Even among the population age 85 years and older, these rates continued to be high (Table 7).

  • Non-malignant meningiomas overall were 2.3 times more common in females compared to males (Fig. 12). Incidence rate ratios were lowest between males and females in persons <20 years old (where incidence rates for males and females were approximately equal), and highest from age 35-54 years, where incidence rates were 3.29 times higher in females (Supplementary Figure 14).

  • Incidence of meningioma was significantly higher in Blacks than in Whites (Fig. 16).

  • The median survival for malignant meningioma was 53 (95% CI: 48-58) months (Table 21). Older adults (40+ years old), Male sex, White race and non-Hispanic ethnicity had poorer survival after diagnosis of malignant meningioma (Table 22).

  • Ten-year relative survival for malignant meningioma was 59.6% (Table 23). Age had a large effect on survival after diagnosis with malignant meningioma: 10-year relative survival was 74.2% for the population age 20-44 years, and 40.8% for age 75+ years (Supplementary Table 13).

  • Ten-year relative survival for non-malignant meningioma was 87.4% (Table 23). Age had a large effect on survival after diagnosis with non-malignant meningioma: 10-year relative survival was 94.5% in AYA, and 81.2% in adults 40+ years old.

  • Site of meningioma had an effect on relative survival after diagnosis with meningioma (Supplementary Figure 15). For non-malignant meningioma, 10-year relative survival was 83.5% for tumors in the cerebral meninges, but 95.8% for tumors in the spinal meninges.

Glioblastoma

  • Glioblastoma was the third most frequently reported CNS histology and the most common malignant tumor overall (Fig. 6B).

  • Glioblastoma accounted for 14.5% of all primary brain and other CNS tumors (Fig. 7B) and 48.6% of primary malignant brain tumors (Fig. 8B).

  • Glioblastoma was more common in older adults (Table 7) and was less common in children (Table 6); these tumors comprised approximately 2.9% of all brain and other CNS tumors reported among age 0-19 years.

  • Incidence of glioblastoma increased with age, with rates highest in individuals age 75 to 84 years (Table 7).

  • Glioblastoma was 1.59 times more common in males (Fig. 12).

  • Glioblastoma was 1.99 times higher among Whites compared to Blacks (Fig. 16).

  • The median survival for glioblastoma for all patients (regardless of treatment) was 8 months (95% CI: 8-9) (Table 21). Older adults (40+ years old), Female sex, White race and non-Hispanic ethnicity were associated with poorer survival after diagnosis of glioblastoma (Table 22). Many other published survival estimates (including many of those previously published by CBTRUS62,63) incorporate treatment patterns which may explain differences between these population-level estimates and other published estimates.

  • Relative survival estimates for glioblastoma were quite low; 7.2% of patients survived five years post-diagnosis (Table 23). These survival estimates were somewhat higher for the small number of patients who were diagnosed under age 20 years (Supplementary Table 13).

Embryonal Tumors

  • Embryonal tumors were the 2nd most frequently reported brain and other CNS tumor histology grouping in children age 0-4 years, and the 5th most common tumor type overall in children and adolescents age 0-19 years (Table 6, Fig. 17B).

  • Embryonal tumors accounted for 12.7% of all primary brain and other CNS tumors in children age 0-14 years (Fig. 18B), 9.9% of tumors in children and adolescents age 0-19 years (Fig. 17B), and 0.90.9% of tumors diagnosed overall (Table 3).

  • Embryonal tumors within the CBTRUS histologic grouping scheme includes multiple different histologies: PNET (ICD-O-3 histology code 9473), medulloblastoma (ICD-O-3 histology codes 9470-9472), ATRT (ICD-O-3 histology code 9508), and several other histologies (Table 2).

  • Incidence of medulloblastoma decreased with age. Incidence was 0.5 per 100,000 population, 0.61 per 100,000 population, 0.34 per 100,000 population, and 0.17 per 100,000 population in children age 0-4, 5-9, 10-14 years, and adolescents age 15-19 years, respectively (Table 6).

  • Incidence of PNET was 0.13 per 100,000 population, 0.05 per 100,000 population, 0.03 per 100,000 population, and 0.03 per 100,000 population in children age 0-4, 5-9, 10-14 years, and adolescents age 15-19 years, respectively (Table 6).

  • Incidence of ATRT was 0.33 per 100,000 population and 0.03 per 100,000 population in children age 0-4 and 5-9 years, respectively. There were too few of these cases in older age groups to report (Table 6).

  • Embryonal tumors were more common in males than females (Fig. 13). This difference was greatest in medulloblastoma, which occurred 1.69 times as frequently in males 0-14 years as compared to females in this age group (Supplementary Fig. 16). Incidence of ATRT and PNET in children 0-14 was not significantly different between males and females.

  • The median survival for embryonal tumors was 66 (CI: 61-73) months (Table 21). Older adults (40+ years old), Black race and Hispanic ethnicity had poorer survival after diagnosis of embryonal tumors. (Table 22).

Descriptive Summary of Incidence Time Trends in Primary Brain and Other CNS Tumors

Time trends in cancer incidence rates are an important measure of the changing burden of cancer in a population over time. Many factors may lead to fluctuations in rates over time, and all of these must be considered when interpreting time trends results. When assessing trends in incidence over time it is critical to use the most recent data available, as delay in reporting may cause small fluctuations in incidence. Time trends analysis methods are used to estimate if the annual percentage change (APC) is significantly different from 0% (meaning no change in incidence from year to year). In addition to assessing statistical significance of changes in incidence over time, the size of this change must also be considered because with datasets as large as CBTRUS very small fluctuations in incidence over time may be statistically significant but not truly represent a large change in proportion of individuals over time.

Incidence rates of cancer overall, and many specific cancer histologies, have decreased over time.64 Overall, changes in incidence rates of all primary brain and other CNS tumors between 2000 and 2017 (limited to 2004 and 2016 for non-malignant tumors), have been small. As stated previously, there are many things that can affect incidence rates over time that are not related to ‘true’ changes in incidence of these tumors such as demographic changes, changes in histologic classification, and changes in cancer registration procedures. The latter is especially applicable to the collection of non-malignant brain and other CNS tumors.

All Malignant Brain and Other CNS Tumors

Please see Fig. 8B for an overview of histologies included in all malignant brain and other CNS tumors.

  • From 2008-2017, there was a slight decrease in overall incidence (APC=-0.8% [95%CI: -1.0%, -0.6%]), Fig. 24, Supplementary Table 15).

  • There was a small but statistically significant increase in incidence in children (age 0-14 years, APC=0.6% [95%CI: 0.4%, 0.9%]), Fig. 24), a small but statistically significant decrease in AYA (age 15-39 years, APC=-0.4% [95%CI: -0.5%, -0.2%]), Fig. 24) from 2000-2017, and a small but statistically significant decrease in older adults from 2007-2017 (age 40+ years, APC=-0.9% [95%CI: -1.1%, -0.8%]), Fig. 24).

Fig. 24.

Fig. 24

Annual Age-Adjusted Incidence Rates of Primary Brain and Other CNS Tumors, and Incidence Trends by Behavior and Age Group, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2000-2017 (varying)

Glioma

Please see Fig. 10B for an overview of histologies included in the broad category of glioma.

  • There was a slight increase in incidence from 2000-2007 (APC=1.1% [95%CI: 0.6%, 1.5%]), Fig. 26), followed by a small but significant decrease in incidence from 2007-2017 (APC=-0.6% [95%CI: -0.8%, -0.3%], Fig. 25).

  • There was a significant increase in incidence in children (age 0-14 years, APC=2.2% [95%CI: 1.5%, 2.9%]) from 2000-2011, and a significant increase in incidence in AYA from 2000-2006 (age 15-39 years, APC=2.5% [95%CI: 1.0%, 3.9%]), Fig. 25).

  • Incidence in older adults (age 40+ years) was relatively stable: there was a statistically significant increase from 2000-2007 (APC=APC=0.6% [95%CI: 0.2%, 1.1%]), followed by a statistically significant decrease from 2007-2017 (APC=0.7% [95%CI: -1.0%, -0.5%], Fig. 25).

  • There was a small but significant increase in incidence of glioblastoma from 2000-2004 (APC=1.1% [95%CI: 0.1, 2.2]), with no significant change between 2007 and 2016 (Fig. 25, Supplementary Table 13).

Fig. 25.

Fig. 25

Annual Age-Adjusted Incidence Rates of Primary Brain and Other CNS Gliomas and Glioblastoma, and Incidence Trends by Age Group, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2000-2017

Malignant Meningioma

  • There was a statistically significant decrease in incidence from 2000-2017 (APC=-4.4% [95%CI: -5.1%, -3.7%]), Supplementary Table 15).

  • Changes were made to histological classification of meningioma in both the 2000 and 2007 revisions of the WHO classification, and gradual uptake of these classification changes may result in changing incidence of these tumors.

All Non-Malignant Brain and Other CNS Tumors

Please see Fig. 9B for an overview of histologies included in all malignant brain and other CNS tumors.

  • There was a significant increase in incidence of non-malignant brain tumors from 2004-2009 (APC=5.2% [95%CI: 3.5%, 7.1%]), Fig. 24, Supplementary Table 16), and no significant change between 2009 and 2016.

  • There was a small but statistically significant increase in incidence of these tumors in children (2004-2014, APC=2.8% [95%CI: 2.0%, 3.6%]), Fig. 24), in AYA (2004-2009, APC=6.5% [95%CI: 3.9%, 9.1%]), Fig. 25), and older adults (2004-2009, APC=5.0% [95% CI: 3.1%, 6.9%], Fig. 24).

  • When analysis was limited to histologically confirmed tumors only, there was a small but significant increase in incidence of non-malignant brain and other CNS tumors from 2004-2009 (APC=1.7% [95%CI: 0.4%, 3.0%]), with no significant change after 2009.

  • There was a statistically significant increase in incidence of radiographically confirmed non-malignant tumors from 2004-2009 (APC=9.6% [95%CI: 6.9%, 12.4%]), with no significant change from 2009-2016.

  • The increases in incidence in the non-malignant tumors are partially attributable to improved collection of radiographically diagnosed cases as well as improvement in collection of non-malignant cases in general over time.

Non-Malignant Meningioma

  • There was a significant increase of non-malignant meningioma from 2004-2008 (APC=6.0% [95%CI: 3.8%, 8.3%]), Fig. 26), but there was no significant change after 2009.

  • When analysis was limited to histologically confirmed cases, there was a slight non-significant increase in incidence from 2004-2008 (APC=1.6% [95%CI: -0.1%, 3.3%]) and there was a slight decrease (APC=-1.2% [95%CI: -1.6%, -0.7%]) from 2008-2017.

  • There was a significant increase in incidence of radiographically diagnosed cases from 2004-2008 (APC=10.6% [95%CI: 7.4%, 13.9%]), and a smaller but still significant change from 2008-2017 (APC=2.0% [95%CI: 1.4%, 2.7%]).

  • The increases in incidence in these non-malignant tumors are partially attributable to improved collection of radiographically diagnosed cases as well as improvement in collection of non-malignant cases in general over time.

Fig. 26.

Fig. 26

Annual Age-Adjusted Incidence Rates of Primary Brain and Other CNS Tumors, and Incidence Trends by Histology for Selected A) Non-Malignant and B) Malignant Histologies, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2000-2017 (varying)

Non-Malignant Nerve Sheath Tumors

  • There was a small but significant increase in the incidence of non-malignant nerve sheath tumors from 2004-2014 (APC=1.8% [95%CI: 1.0%, 2.6%]), Supplementary Table 16).

  • When analysis was limited to histologically confirmed cases only, there was a significant decrease in incidence (APC=-1.7% (95%CI: -3.3%, -0.2%]) from 2004-2010.

  • There was a significant increase in incidence of radiographically diagnosed tumors from 2004-2007 (APC=8.7% [95%CI: 3.3%, 14.4%]) and 2007-2014 (APC=2.9% [95%CI: 1.4%, 4.4%]).

  • The increases in incidence in these non-malignant tumors are partially attributable to improved collection of radiographically diagnosed cases as well as improvement in collection of non-malignant cases in general over time.

Vestibular Schwannoma

Vestibular schwannoma (Table 2) is the most common type of nerve sheath tumor, representing 75% of all non-malignant nerve sheath tumors (Fig. 9B).

  • There was a small but significant increase in the incidence of non-malignant nerve sheath tumors from 2004-2014 (APC=1.7% [95%CI: 1.0%, 2.5%]), Fig. 26), with a decrease from 2014-2017 (APC=-4.5% [95%CI:-8.8%, -0.0%]).

  • When analysis was limited to histologically confirmed cases only, there was a significant decrease in incidence (APC=-1.3% (95%CI: -1.9%, -0.8%]) from 2004-2017.

  • There was a significant increase in incidence of radiographically diagnosed tumors from 2004-2007 (APC=9.5% [95%CI: 3.6%, 15.7%]) and 2007-2014 (APC=3.0% [95%CI: 1.4%, 45%]), with a significant decrease from 2014-2017 (APC=-4.2% [95%CI: -8.3%, 0.0%]).

  • The increases in incidence in these non-malignant tumors are partially attributable to improved collection of radiographically diagnosed cases as well as improvement in collection of non-malignant cases in general over time.

Non-Malignant Tumors of the Pituitary

  • There was a significant increase in non-malignant tumors of the pituitary from 2004-2008 (APC=7.8% [95%CI: 5.5%, 10.1%]), Fig. 26), and 2008-2012 (APC=3.3% [95%CI: 0.3%, 6.3%]), Fig. 27A) but no significant change in incidence from 2008-2016.

  • When analysis was limited to histologically confirmed tumors only, there was a significant increase (APC=4.6% [95%CI: 3.2%, 6.0%]) from 2004-2009).

  • There was a significant increase in incidence of radiographically diagnosed tumors of the pituitary from 2004-2008 (APC=11.5% [95%CI: 7.3%, 15.8%]) and 2008-2012 (APC=6.9% [95%CI: 1.9%, 12.1%]), with no significant change in incidence after 2012.

Prevalence of Primary Malignant Brain and Other CNS Tumors

Prevalence is an estimate of the total number of individuals with a disease who currently are alive within a population, as compared to incidence, which is a calculation based on new diagnoses only. These calculations take into account not only the number of new cases being diagnosed, but also the length of time that individuals survive after diagnosis. CBTRUS previously estimated the 2010 point prevalence rate for all primary malignant brain and other CNS tumors to be 47.6 per 100,000 population, or a total of 103,634 cases.65 Prevalence in children (0-14 years old) was estimated to be 22.31 per 100,000 population (13,657 cases), while prevalence in AYA (15-39 years old) was estimated to be 48.49 per 100,000 (31,299 cases). These ages represent age at time of prevalence calculation and not the age at which individuals were diagnosed. Please refer to Zhang, et al65 for more details.

CBTRUS also previously estimated the 2014 prevalence of selected adult malignant brain tumor histologies. Glioblastoma had the highest prevalence, at 9.23 per 100,000 population (23,327 cases), followed by diffuse astrocytoma (4.68 per 100,000 population; 10,868 cases), and oligodendroglioma (3.57 per 100,000 population; 8,217 cases). Please refer to Gittleman, et al66 for more details, including sex-, race-, and ethnicity-specific prevalence estimates.

Lifetime Risk of Primary Malignant Brain and Other CNS Tumors

From birth, a person in the US has a 0.62% chance of ever being diagnosed with a primary malignant brain and other CNS tumor (excluding lymphomas, leukemias, tumors of the pituitary and pineal glands, and olfactory tumors of the nasal cavity) and a 0.48% chance of dying from a primary malignant brain/other CNS tumor.67-70

  • For males (all races), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.69% and 0.54%, respectively.

  • For females (all races), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.55% and 0.42%, respectively.

  • For White non-Hispanics (both sexes), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.72% and 0.55%, respectively.

  • For White Hispanics (both sexes), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.55% and 0.40%, respectively.

  • For Blacks (both sexes), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.33% and 0.26%, respectively.

  • For API (both sexes), the risk of developing and the risk of dying from a primary malignant brain and other CNS tumor is 0.41% and 0.32%, respectively.

Risk Factors for Primary Brain and Other CNS Tumors

Many environmental and behavioral risk factors have been investigated for primary brain and other CNS tumors. The only well-validated risk factors for these tumors (particularly meningiomas) is an increased risk with exposure to ionizing radiation71 (the type of radiation generated by atomic bombs, therapeutic radiation treatment, and some forms of medical imaging) and a decreased risk for these tumors (particularly glioma) in persons with a history of allergy or other atopic disease72 (including eczema, psoriasis, and asthma). Having a first-degree family member (including parents, children, and full siblings) that has been diagnosed with a brain tumor has been shown to increase risk approximately two-fold.73-78 Several recent review articles have elaborated on the current state of risk factor research in primary brain and other CNS tumors.79-82

Biomarkers for Primary Brain and Other CNS Tumors

Primary brain and other CNS tumors are a highly heterogeneous group of diseases, and characterization of unique tumor histologies within this group has been refined over time. The development of technologies for characterizing DNA, RNA, and DNA methylation has led to the discovery of several factors (known as ‘biomarkers’) that can be used to more accurately classify these tumors than histologic appearance alone. See Table 24 for a brief overview of selected biomarkers for primary brain and other CNS tumors, as well as a more in depth discussion in Scheie, et al83 and Velázquez Vega and Brat84, as well as in Guerreiro Stucklin, et al.85 for discussion on pediatric biomarkers specifically.

Table 24.

Summary of Biomarkers Identified for Primary Brain and Other CNS Tumors as Collected in Central Cancer Registries Starting with the 2018 Diagnosis Yeara.

Histology Gene or Marker Outcome Related scientific publications Collected by US Cancer Registry System
Glioma (especially oligodendroglial tumors) Large deletions (missing parts of the chromosome) in the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q) Improved response to chemotherapy and radiation, and increased survival Cairncross JG, Ueki K et al. (1998)
Vogelbaum MA, Hu C et al. (2015)
van den Bent MJ, Brandes AA et al. (2013)
The Cancer Genome Atlas Research Network, Brat DJ et al. (2015)
Ceccarelli M, Barthel FP et al. (2016)
Yes
Site-specific factor 5: http://web2.facs.org/cstage0205/brain/Brain_nph.html
Site-specific factor 6: http://web2.facs.org/cstage0205/brain/Brain_opf.html
Glioma (especially low grade astrocytomas and oligodendroglial tumors) Protein-truncating mutation in isocitrate dehydrogenase 1 (IDH1) or in isocitrate dehydrogenase 2 (IDH2) Increased survival time Yan H, Parsons DW et al. (2009)
The Cancer Genome Atlas Research Network, Brat DJ et al. (2015)
Ceccarelli M, Barthel FP et al. (2016)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816
Glioma (especially IDH mutated glioma) Loss of function mutation in alpha thalassemia/mental retardation syndrome X‐linked (ATRX) Increased survival time Jiao Y, Killela PJ et al. (2012)
Wiestler B, Capper D et al. (2013)
The Cancer Genome Atlas Research Network, Brat DJ et al. (2015)
No
Glioblastoma Methylation of the promoter of O-6-methylguanine-DNA methyltransferase (MGMT) Limits ability of the tumor cells to repair DNA damage caused by chemotherapy and radiation; results in increased survival time Hegi ME, Diserens AC et al. (2005)
Stupp R, Hegi ME et al. (2007)
Hegi ME, Liu L et al. (2008)
Yes
Site-specific factor 4: http://web2.facs.org/cstage0205/brain/Brain_mpn.html
Glioblastoma Glioma-CpG island methylator phenotype (G-CIMP), Genome-wide DNA methylation Significantly increased survival time Noushmehr H, Weisenberger DJ et al. (2010) No
Glioblastoma Amplification of epidermal growth factor receptor (EGFR) Activates the RTK/RAS/PI3K pathway, leading to increased proliferation. Associated with poorer survival. Maire CL and Ligon KL (2014)
Ceccarelli M, Barthel FP et al. (2016)
No
Glioma (oligodendroglial tumors and IDH wild type glioblastoma) Mutation of promotor of Telomerase reverse transcriptase (TERT) Facilitates increased telomere lengthening, and decreases survival in IDH wild type glioma Arita H, Narita Y et al. (2013)
Eckel-Passow JE, Lachance DH et al. (2015)
Ceccarelli M, Barthel FP et al. (2016)
No
Glioma (particularly pediatric lower grade gliomas) Mutation or fusion of B-Raf (BRAF) Activates the RAS/MAPK pathway. Fusion leads to improved survival. Hawkins C, Walker E et al. (2011) No
Medulloblastoma Wingless (WNT) subtype Low prevalence of metastatic disease (~5-10%)
Highest five-year survival (~95%)
Kool M, Korshunov A et al. (2012)
Northcott PA, Dubuc AM et al. (2012)
Northcott PA, Jones DT et al. (2012)
Northcott PA, Buchhalter I et al. (2017)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816
Medulloblastoma Sonic hedgehog subtype (SHH) Moderate prevalence of metastatic disease (~15-20%)
Moderate five-year survival (~75%)
Kool M, Korshunov A et al. (2012)
Northcott PA, Dubuc AM et al. (2012)
Northcott PA, Jones DT et al. (2012)
Northcott PA, Buchhalter I et al. (2017)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816
Medulloblastoma Group 3 subtype (also known as Group C) Increased prevalence of metastatic disease (~40-45%)
Poorest five-year survival (~50%)
Kool M, Korshunov A et al. (2012)
Northcott PA, Dubuc AM et al. (2012)
Northcott PA, Jones DT et al. (2012)
Northcott PA, Buchhalter I et al. (2017)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816
Medulloblastoma Group 4 subtype (also known as Group D) Increased prevalence of metastatic disease (40-45%)
Moderate five-year survival (~75%)
Kool M, Korshunov A et al. (2012)
Northcott PA, Dubuc AM et al. (2012)
Northcott PA, Jones DT et al. (2012)
Northcott PA, Buchhalter I et al. (2017)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816
Embryonal tumor C19MC amplification and presence of multilayered rosettes Highly aggressive, with average survival of 12 months after diagnosis Ceccom J, Bourdeaut F et al. (2014)
Korshunov A, Sturm D et al. (2014)
Yes
Began in collection year 2018 (January 1), http://datadictionary.naaccr.org/default.aspx?c=10#3816

a. Available to CBTRUS for the first time in 2021.

Arita, H., Y. Narita, S. Fukushima, et al. Upregulating mutations in the TERT promoter commonly occur in adult malignant gliomas and are strongly associated with total 1p19q loss. Acta Neuropathol. 2013;126(2):267-276. PMID: 23764841. DOI: 10.1007/s00401-013-1141-6.

Cairncross, J. G., K. Ueki, M. C. Zlatescu, et al. Specific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomas. J. Natl Cancer Inst. 1998;90(19):1473-1479. PMID: 9776413.

Ceccarelli, M., F. P. Barthel, T. M. Malta, et al. Molecular Profiling Reveals Biologically Discrete Subsets and Pathways of Progression in Diffuse Glioma. Cell. 2016;164(3):550-563. PMID: 26824661. PMCID: 4754110. DOI: 10.1016/j.cell.2015.12.028.

Ceccom, J., F. Bourdeaut, N. Loukh, et al. Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. Clin. Neuropathol. 2014;33(1):15-22. PMID: 23863344. DOI: 10.5414/np300636.

Eckel-Passow, J. E., D. H. Lachance, A. M. Molinaro, et al. Glioma Groups Based on 1p/19q, IDH, and TERT Promoter Mutations in Tumors. N. Engl. J. Med. 2015;372(26):2499-2508. PMID: 26061753. PMCID: 4489704. DOI: 10.1056/NEJMoa1407279.

Hawkins, C., E. Walker, N. Mohamed, et al. BRAF-KIAA1549 fusion predicts better clinical outcome in pediatric low-grade astrocytoma. Clin. Cancer Res. 2011;17(14):4790-4798. PMID: 21610142. DOI: 10.1158/1078-0432.ccr-11-0034.

Hegi, M. E., A. C. Diserens, T. Gorlia, et al. MGMT gene silencing and benefit from temozolomide in glioblastoma. N. Engl. J. Med. 2005;352(10):997-1003. PMID: 15758010. DOI: 10.1056/NEJMoa043331.

Hegi, M. E., L. Liu, J. G. Herman, et al. Correlation of O6-methylguanine methyltransferase (MGMT) promoter methylation with clinical outcomes in glioblastoma and clinical strategies to modulate MGMT activity. J. Clin. Oncol. 2008;26(25):4189-4199. PMID: 18757334. DOI: 10.1200/JCO.2007.11.5964.

Jiao, Y., P. J. Killela, Z. J. Reitman, et al. Frequent ATRX, CIC, FUBP1 and IDH1 mutations refine the classification of malignant gliomas. Oncotarget. 2012;3(7):709-722. PMID: 22869205. PMCID: PMC3443254. DOI: 10.18632/oncotarget.588.

Kool, M., A. Korshunov, M. Remke, et al. Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas. Acta Neuropathol. 2012;123(4):473-484. PMID: 22358457. PMCID: 3306778. DOI: 10.1007/s00401-012-0958-8.

Korshunov, A., D. Sturm, M. Ryzhova, et al. Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 2014;128(2):279-289. PMID: 24337497. DOI: 10.1007/s00401-013-1228-0.

Maire, C. L. and K. L. Ligon. Molecular pathologic diagnosis of epidermal growth factor receptor. Neuro. Oncol. 2014;16 Suppl 8:viii1-6. PMID: 25342599. PMCID: PMC4207139. DOI: 10.1093/neuonc/nou294.

Northcott, P. A., I. Buchhalter, A. S. Morrissy, et al. The whole-genome landscape of medulloblastoma subtypes. Nature. 2017;547(7663):311-317. PMID: 28726821. PMCID: PMC5905700. DOI: 10.1038/nature22973.

Northcott, P. A., A. M. Dubuc, S. Pfister, et al. Molecular subgroups of medulloblastoma. Expert Rev. Neurother. 2012;12(7):871-884. PMID: 22853794. PMCID: 4334443. DOI: 10.1586/ern.12.66.

Northcott, P. A., D. T. Jones, M. Kool, et al. Medulloblastomics: the end of the beginning. Nat. Rev. Cancer. 2012;12(12):818-834. PMID: 23175120. PMCID: 3889646. DOI: 10.1038/nrc3410.

Noushmehr, H., D. J. Weisenberger, K. Diefes, et al. Identification of a CpG island methylator phenotype that defines a distinct subgroup of glioma. Cancer Cell. 2010;17(5):510-522. PMID: 20399149. PMCID: 2872684. DOI: 10.1016/j.ccr.2010.03.017.

Stupp, R., M. E. Hegi, M. R. Gilbert, et al. Chemoradiotherapy in malignant glioma: standard of care and future directions. J. Clin. Oncol. 2007;25(26):4127-4136. PMID: 17827463. DOI: 10.1200/JCO.2007.11.8554.

The Cancer Genome Atlas Research Network, D. J. Brat, R. G. Verhaak, et al. Comprehensive, Integrative Genomic Analysis of Diffuse Lower-Grade Gliomas. N. Engl. J. Med. 2015;372(26):2481-2498. PMID: 26061751. PMCID: 4530011. DOI: 10.1056/NEJMoa1402121.

van den Bent, M. J., A. A. Brandes, M. J. Taphoorn, et al. Adjuvant procarbazine, lomustine, and vincristine chemotherapy in newly diagnosed anaplastic oligodendroglioma: long-term follow-up of EORTC brain tumor group study 26951. J. Clin. Oncol. 2013;31(3):344-350. PMID: 23071237. DOI: 10.1200/JCO.2012.43.2229.

Vogelbaum, M. A., C. Hu, D. M. Peereboom, et al. Phase II trial of pre-irradiation and concurrent temozolomide in patients with newly diagnosed anaplastic oligodendrogliomas and mixed anaplastic oligoastrocytomas: long term results of RTOG BR0131. J. Neuro-Oncol. 2015;124(3):413-420. PMID: 26088460. PMCID: 4584176. DOI: 10.1007/s11060-015-1845-7.

Wiestler, B., D. Capper, T. Holland-Letz, et al. ATRX loss refines the classification of anaplastic gliomas and identifies a subgroup of IDH mutant astrocytic tumors with better prognosis. Acta Neuropathol. 2013;126(3):443-451. PMID: 23904111. DOI: 10.1007/s00401-013-1156-z.

Yan, H., D. W. Parsons, G. Jin, et al. IDH1 and IDH2 mutations in gliomas. N. Engl. J. Med. 2009;360(8):765-773. PMID: 19228619. PMCID: 2820383. DOI: 10.1056/NEJMoa0808710.

Gliomas, as the most common malignant primary brain and other CNS tumor type, have been subject to the greatest investigation. A recent review has described in detail the current state of glioma biomarker research.86 One of the earliest discoveries in glioma biomarkers was that oligodendroglioma often had large deletions (missing parts of the chromosome, also known as loss of heterozygosity) in the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q).87 In general, these deletions significantly predict positive response to chemotherapy and radiation treatment in oligodendroglioma and anaplastic oligodendroglioma.88-90 Mutations to the genes in isocitrate dehydrogenase 1 (IDH1) and in isocitrate dehydrogenase 2 (IDH2) have also been shown to be associated with improved prognosis in glioma.91-93 These mutations are common in lower grade gliomas (WHO grade II and WHO grade III), but are rare in glioblastoma.92 Both of these alterations are thought to occur relatively early in the development of gliomas; the prevalence of this mutation varies by anatomic location in the brain.94,95 The combination of these two factors can be used to more accurately stratify glioma by prognosis than the previously utilized histological criteria,93,96 and have been incorporated into the definition of oligodendroglioma and astrocytoma in the 2016 update to the WHO classification.21These classification changes are not reflected in the data presented in this report, which were collected prior to the adoption of these biomarkers as diagnostic criteria. These new biomarkers began to be collected by CCRs in the US starting January 1, 2018 and will be available to CBTRUS for the first time with the 2021 NPCR and SEER data releases.

Another alteration that is associated with improved survival in glioma is increased methylation (where methyl molecules are bonded to the DNA) of the promotor region of the gene O-6-methylguanine-DNA methyltransferase (MGMT).97,98 The promoter region of a gene is located upstream of the coding part of the gene and exerts control over whether a gene is transcribed into RNA. Methylation of this region effectively silences the gene, and prevents transcription into RNA. MGMT is a DNA repair protein, and it is assumed that the decreases in protein levels increase sensitivity to the alkylating chemotherapies (e.g. temozolomide) often used in the treatment of gliomas aimed to combat tumor growth through DNA damage.99 This alteration is common in glioblastoma and less common in lower grade gliomas. Recent analyses of data generated by The Cancer Genome Atlas (TCGA) have shown that genome-wide DNA methylation predicts improved prognosis in addition to methylation of specific genes.96 Persons whose tumor has a higher proportion of methylation across the genome are termed to have glioma-CpG island methylator phenotype (G-CIMP).100 G-CIMP and MGMT methylation are correlated,101 but G-CIMP is much rarer in glioblastoma than MGMT methylation.

Medulloblastoma is another tumor type that has been subject to significant molecular analysis. Using an analysis of gene expression (based on quantity of RNA transcribed from a gene), medulloblastoma was able to be subdivided into four distinct subtypes: wingless (WNT), sonic hedgehog (SHH), group 3 (also called group C), and group 4 (also called group D).102 These groups are associated with specific age-groups, with SHH being most common in infants and adults, and all other groups being more common in childhood. Several review articles have elaborated on the details of these subgroups and their implications for diagnosis and treatment.103-105

Diffuse intrinsic pontine glioma (DIPG) is an aggressive tumor of the brain stem that occurs primarily in children, and accounts for ~75% of brain stem tumors in children. Survival is very poor after diagnoses with these tumors. Due to the location of these tumors, they are often not biopsied and, therefore, have not been molecularly characterized to the extent of many other primary brain and other CNS tumor types. Recently, biopsy and autopsy protocols have allowed for collection of primary tumor samples that have been used for genomic profiling.106-108 These tumors have been found to be highly heterogeneous. Mutations in histone H3, Activin A receptor, type I (ACVR1), tumor protein p53 (TP53), platelet-derived growth factor receptor A (PDGFRA), phosphatidylinositol 3-kinase catalytic subunit alpha (PIK3CA), and Myc (MYC) have been identified as characteristic of these tumors.107,109,110 A recent review has further summarized recent developments in the genomics of DIPG.111

As of 2011, SEER registries currently collect information on three validated biomarkers for primary brain and other CNS tumors as Site Specific Factors (SSF): promoter methylation status of MGMT (SSF 4), deletion of the 1p (SSF 5), and deletion of 19q (SSF 6).112 Completeness of these biomarker data varies significantly by histology, but is gradually improving over time.

Starting with diagnosis year 2018, the US cancer registry system began collecting information on multiple brain and other CNS markers, including IDH1/2 mutation, 1p/19q codeletion, medulloblastoma molecular subtypes, and all biomarkers found in 2016 WHO classification. These data will be available to CBTRUS for the first time with the 2021 NPCR and SEER data releases for the 2018 diagnosis year only.

Strengths and Limitations of Cancer Registry Data

CBTRUS, in collaboration with the CDC and NCI, is the largest population-based registry focused exclusively on primary brain and other CNS tumors in the US and represents cases collected from the entire US population. The CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017 contains the most up-to-date population-based data on all primary brain tumor and other CNS tumors available through the cancer surveillance system in the US.

Registration of individual cases is conducted by cancer registrars at the institution where diagnosis or treatment occurs and is then transmitted to the central cancer registry, which further transmits this information to NPCR and/or SEER. CCRs, both NPCR and SEER, only report cases to the CDC and NCI for persons who are residents of that particular state, so duplicate records should not occur for persons who may have traveled across state lines for treatment. As a result, the CBTRUS dataset is a complete recording of all cases for the time period examined with minimal duplicates.

Currently, there is no publicly available data source for the collection of survival and outcomes data from all geographic regions in the US via the cancer registry system. Survival data used for this report are collected by NPCR for 45 of the 51 CCR in the US—primarily through linkage with death certificate and other administrative records—and by SEER for the remaining CCR—through both active and passive methods—and the feasibility of these data for use in survival studies has been evaluated113,114 and shown to produce reliable and robust estimates of cancer survival. Use of passive follow-up with record linkage may result in overestimation of survival in some populations, such as those that are more likely to leave the state or country.

No mechanism currently exists for central pathology review of cases within the US cancer registry system, and histology code assignment at case registration is based on histology information contained in the patient’s medical record. The WHO Classification of Tumours of the Central Nervous System underwent revision in 1993,115 2000,20 2007,2 and 2016.21 As of 2018, the US cancer registry system is using the 2016 classification for data abstraction, but tumors included in this report may have been diagnosed using any of the available classifications prior to 2013 due to the variation in adoption of new standards by individual physicians and medical practices. As a result, histologies are reflective of the prevailing criteria for the histology at the time of case registration. This means that despite changes to the histology schema that may occur over time, it is not possible, without additional variables, to go back and reclassify tumors based on the new criteria. In addition to changes in histologic criteria over time, there is significant inter-rater variability in histopathological diagnosis of glioma.116,117 This also means that incomplete, incorrect, or alternatively stated diagnoses included in a pathology report or other medical record may result in an incorrect reporting of the details of an individual case. For example, an anaplastic oligodendroglioma recorded in a pathology record as oligodendroglioma WHO grade III may be incorrectly recorded as an oligodendroglioma when the accurate category is an anaplastic oligodendroglioma.

US cancer registration requires the reporting of cases that are confirmed by different types of diagnostic procedures, including both histologic confirmation (where surgery was performed and the diagnosis confirmed by a pathologist) and radiographic confirmation (where diagnosis was made based solely on imaging criteria, such as an MRI, CT scan, or X-ray). Only histologic confirmation allows certainty on the assignment of a specific histology as well as for an assignment of a WHO grade. Many tumors have unique characteristics that make them identifiable on imaging, and thereby qualify as a valid type of diagnostic procedure, but it is important to consider the decreased level of certainty of specifying the correct histology in these tumors.

The 2016 WHO Classification of Tumours of the Central Nervous System21 contains significant updating to diagnostic criteria for glioma. Oligoastrocytoma has long been considered an entity that is distinct from astrocytoma and oligodendroglioma, and is included as a unique histologic grouping within the CBTRUS classification scheme. Recent molecular analyses suggest that these tumors are not molecularly distinct from oligodendrogliomas or astrocytomas118 and can be separated into astrocytoma or oligodendroglioma using molecular markers; the diagnosis of oligoastrocytoma is strongly discouraged and is qualified with a “not otherwise specified” (NOS) designation under the 2016 WHO Classification of Tumours of the Central Nervous System. With this recent updating to the WHO criteria for central nervous system tumors,21IDH1/2 mutation and 1p/19q co-deletion will become the primary factors by which gliomas are classified. While data on IDH1/2 mutation status was not collected in the US cancer registry system during the time period covered by this report, these data are required to be collected by cancer registrars (as available in the medical record) as of January 1, 2018. Cancer registry systems have collected 1p/19q deletion data for some of the report years, but data vary significantly in completeness by histology.112 It is likely that these changes to diagnostic criteria may affect the incidence of these tumor types in future years of the CBTRUS Statistical Report.

Concluding Comment

The CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2013-2017 comprehensively describes the most up-to-date- (October 2020) population-based incidence, mortality, observed and relative survival of primary malignant and non-malignant brain and other CNS tumors collected and reported by central cancer registries covering the entire US population. This report aims to serve as a useful resource for researchers, clinicians, patients, and families. In keeping with its mission, CBTRUS continually revises its reports to reflect the current collection and reporting practices of the broader surveillance community in which it works, while integrating the input it receives from the clinical and research communities, especially from neuropathologists, when possible. In this way, CBTRUS facilitates communication between the cancer surveillance and the brain tumor research and clinical communities and contributes meaningful insight into the descriptive epidemiology of all primary brain and other CNS tumors in the US.119

Supplementary Material

noaa200_suppl_Supplementary_Materials

Acknowledgments

This report was prepared by the CBTRUS Scientific Principal Investigator, Jill Barnholtz-Sloan, Ph.D., her research staff affiliated with the Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Quinn Ostrom, Ph.D., M.P.H. from Baylor College of Medicine, and CBTRUS President and Chief Mission Officer Carol Kruchko. The CBTRUS data presented in this report were provided through an agreement with the CDC, NPCR. In addition, CBTRUS used data from the research data files of the NCI, SEER Program. CBTRUS acknowledges and appreciates these contributions to this report and to cancer surveillance in general.

We acknowledge the efforts of the tumor registrars at hospitals and treatment centers, the CCRs, and the staff from the NPCR and SEER programs, whose efforts to collect accurate and complete data have made this report possible. We are also grateful to the four neuropathologists, Drs. Janet Bruner, Roger McLendon, Tarik Tihan, and Daniel Brat, who reviewed the CBTRUS histology grouping scheme, to our Consulting Neuropathologist, Dr. Janet Bruner, who answered our questions and provided feedback throughout the year, to our Board of Directors and Advisors, and especially Hoda Anton-Culver, Ph.D., Elizabeth Claus, M.D.,Ph.D., Roberta McKean-Cowdin, Ph.D., Nancy Stroup, Ph.D., and Reda J, Wilson, M.P.H., C.T.R. who reviewed this report.

Glossary

Abbreviations

AAAIR

Average Annual Age-Adjusted Incidence Rate

AAAMR

Average Annual Age-Adjusted Mortality Rate

ABTA

American Brain Tumor Association

ACVR1

Activin A receptor type I

AIAN

American Indian/Alaskan Native

AJCC

American Joint Commission on Cancer

APC

Annual Percent Change

API

Asian or Pacific Islander

AYA

Adolescents and Young Adults

ATRT

Atypical Teratoid Rhabdoid Tumor

CBTRUS

Central Brain Tumor Registry of the United States

CCR

Central Cancer Registry

CDC

Centers for Disease Control and Prevention

CS

Collaborative Staging

CI

Confidence Interval

CNS

Central Nervous System

DIPG

Diffuse Intrinsic Pontine Glioma

G-CIMP

glioma-CpG island methylator phenotype

ICD-O-3

International Classification of Diseases for Oncology, Third Edition

ICCC

International Classification of Childhood Cancer

IDH1/2

Isocitrate Dehydrogenase ½

MGMT

O-6-Methylguanine-DNA Methyltransferase

NAACCR

North American Association of Central Cancer Registries

NCHS

National Center for Health Statistics

NCI

National Cancer Institute

NOS

Not Otherwise Specified

NPCR

National Program of Cancer Registries

NPCR-CSS

NPCR Cancer Surveillance System

NVSS

National Vital Statistics System

PDGFRA

Platelet-derived Growth Factor Receptor A

PI3KCA

Phosphatidylinositol 3-Kinase Catalytic subunit Alpha

PNET

Primitive Neuroectodermal Tumor

SEER

Surveillance Epidemiology and End Results

SHH

Sonic Hedgehog

SSF

Site Specific Factors

TCGA

The Cancer Genome Atlas

TP53

Tumor Protein p53

RUCC

Rural Urban Continuum Codes

UDS

Uniform Data Standards

US

United States

USCS

United States Cancer Statistics

VACCR

Veterans Affairs Central Cancer Registry

VHA

Veteran’s Health Administration

WHO

World Health Organization

WNT

Wingless

Supplementary Material (Online Only)

Supplementary Table 1. Main and Extended Classification for ICCC Recode ICD-O-3/WHO 2008, based on WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues (2008), for Selected Histologies1,2

Supplementary Table 2. 2000 U.S. Standard Population

Supplementary Table 3. Average Annual Populationa for 51 Central Cancer Registries (Including 50 States and District of Columbia) for 2013-2017 by Age, Sex, and Race

Supplementary Table 4. Average Annual Populationa for 51 Central Cancer Registries (Including 50 States and District of Columbia) for 2013-2017 by Age, Sex, and Hispanic Ethnicity

Supplementary Table 5. Average Annual Populationa for 51 Central Cancer Registries (Including 50 States and District of Columbia) for 2013-2017 by Age, Sex, and Urban/Rural Location of Residenceb

Supplementary Table 6. Five-Year Total, Average Annual Totala and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Overall Cancer Incidence, and the Top Fifteen Most Incident Comparison Cancers by NCI Age Group, CBTRUS Statistical Report: NPCR and SEER, 2013-2017

Supplementary Table 7. Five-Year Total, Average Annual Totala, and Average Annual Age-Adjusted Mortality Ratesb with 95% Confidence Intervals by Cause of Death and NCI Age Group, United States, NVSS, 2013-2017

Supplementary Table 8. Five-Year Total, Annual Average Totala, and Age-Adjusted and Age-Specific Incidence Ratesb with 95% Confidence Intervals for Children and Adolescents (Age 0-19 Years), Brain and Other Central Nervous System Tumors: Malignant and Non-Malignant by International Classification of Childhood Cancer (ICCC), CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Table 9. Five-Year Total, Average Annual Totala, and Average Annual Age-Adjusted Incidence Ratesb with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Behavior and Urban/Rural Location of Residence, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Table 10. Estimated Number of Casesa,b of Brain and Other Central Nervous System Tumors Overall and by Age, Major Histology Grouping, and Histology, 2020, 2021

Supplementary Table 11. Five-Year Total, Average Annual Totala, and Average Annual Age-Adjusted Mortality Ratesb with 95% Confidence Intervals for Malignant Brain and Other Central Nervous System Cancer Overall and by State and Urban/Rural Location of Residencec, United States, 2013-2017

Supplementary Table 12. One-, Five-, and Ten-Year Relative Survival Ratesa,b with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Primary Site, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR, 2004-2016c

Supplementary Table 13. One-, Five-, and Ten-Year Relative Survival Ratesa,b with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Age Group, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR, 2004-2016c

Supplementary Table 14. One-, Five-, and Ten-Year Relative Survival Ratesa,b with 95% Confidence Intervals for Brain and Other Central Nervous System Tumors by Histology, Behavior and Urban/Rural Location of Residencec, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR, 2004-2016

Supplementary Table 15. Annual Percent Change (APC) and 95% Confidence Intervals for Malignant Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Behavior, and Sex, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR and SEER, 2000-2017

Supplementary Table 16. Annual Percent Change (APC) and 95% Confidence Intervals for Non-Malignant Brain and Other Central Nervous System Tumors by Major Histology Grouping, Histology, Behavior, and Sex, CBTRUS Statistical Report: U.S. Cancer Statistics - NPCR and SEER, 2004-2017

Supplementary Fig. 1. Average Annual Age-Adjusted Incidence Ratesa with 95% Confidence Intervals of All Primary Brain and Other CNS Tumors in Comparison To Top Five Common Causes of Cancer Death and Top Three Non-Cancer Causes of Death for Children Age 0-14 Years, Adolescents and Young Adults Age 15-39 Years, and Older Adults Age 40+ Years in A) Males and B) Females, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER 2013-2017

Supplementary Figure 2. Average Annual Age-Adjusted Mortality Ratesa with 95% Confidence Intervals of All Primary Brain and Other CNS Tumors in Comparison To Top Five Common Causes of Cancer Death and Top Three Non-Cancer Causes of Death for Children Age 0-14 Years, Adolescents and Young Adults Age 15-39 Years, and Older Adults Age 40+ Years in A) Males and B) Females, CBTRUS Statistical Report: NVSS, 2013-2017

Supplementary Figure 3. Distributiona of A) Malignant Primary Brain and Other CNS Tumors (Five-Year Total=123,484; Annual Average Cases=24,697) and B) Non-Malignant Primary Brain and Other CNS Tumors (Five-Year Total=291,927; Annual Average Cases=58,385) by Major Histology Groups, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Supplementary Figure 4 Annual Age-Adjusted Incidence Ratesa of Primary Brain and Other CNS Tumors by Year and Behavior, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 5. Annual Age-Adjusted Incidence Ratesa of Primary Brain and Other CNS Tumors by Year and Behavior, in A) Males and B) Females, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Supplementary Figure 6. Distributiona of Schwannoma (9560/0) by Site (Five-Year Total=33,856; Annual Average Cases=6,771), CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 7. Distributiona of Primary Brain and Other CNS Tumors, Males Only, Overall (Five-Year Total=173,641; Annual Average Cases=34,728), by A) Site and B) Histology, Malignant (Five-Year Total=68,578; Annual Average Cases=13,716), by C) Site and D) Histology, and Non- Malignant (Five-Year Total=105,063; Annual Average Cases=21,013), by E) Site and F) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 8. Distributiona of Primary Brain and Other CNS Tumors, Females Only, Overall (Five-Year Total=173,641; Annual Average Cases=48,354), by A) Site and B) Histology, Malignant (Five-Year Total=54,906; Annual Average Cases=10,981), by C) Site and D) Histology, and Non-Malignant (Five-Year Total=186,864; Annual Average Cases=37,373), by E) Site and F) Histology, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 9. Average Annual Age-Adjusted Incidence Ratesa of Primary Brain and Other CNS Tumors by Age and Behavior, (A) Males, and (B) Females, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Supplementary Figure 10. Average Annual Age-Adjusted Incidence Ratesa with 95% Confidence Intervals of Selected Primary Brain and Other CNS Tumor Histologies by Sex, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 11. Average Annual Age-Adjusted Incidence Ratesa of Malignant and Non-Malignant Primary Brain and Other CNS Tumors Combined by Central Cancer Registry, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Supplementary Figure 12. Distributiona of Primary Brain and Other CNS Tumors Diagnosed in Puerto Rico by Behavior (Five-Year Total=2,356; Annual Average Cases=471), CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR, 2013-2017

Supplementary Figure 13. Incidence Rate Ratios by Ethnicity (Non-Hispanic:Hispanic) for Selected Primary Brain and Other CNS Tumor Histologies, CBTRUS Statistical Report: US Cancer Statistics - NPCR and SEER, 2013-2017

Supplementary Figure 14. Incidence Rate Ratios for Meningioma with 95% Confidence Intervals by Behavior, Sex (Males:Females), and Age Group, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

Supplementary Figure 15. Relative Survival Rates for Meningioma by Behavior and Site, CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR, 2004-2016

Supplementary Figure 16. Incidence Rate Ratios in Children (Age 0-14 Years) for Selected Embryonal Histologies by Sex (Males:Females), CBTRUS Statistical Report: U.S. Cancer Statistics – NPCR and SEER, 2013-2017

CBTRUS Mission

CBTRUS is a not-for-profit corporation committed to providing a resource for gathering and disseminating current epidemiologic data on all primary brain and other central nervous system tumors, malignant and non-malignant, for the purposes of accurately describing their incidence and survival patterns, evaluating diagnosis and treatment, facilitating etiologic studies, establishing awareness of the disease, and ultimately, for the prevention of all brain tumors.

Disclaimer

CBTRUS is a not-for-profit corporation which gathers and disseminates epidemiologic data on primary brain and other central nervous system tumors in order to facilitate research and establish awareness of the disease. CBTRUS makes no representations or warranties, and gives no other assurances or guarantees, express or implied, with respect to the accuracy or completeness of the data presented. The information provided in this report is not intended to assist in the evaluation, diagnosis, or treatment of individual diseases. Persons with questions regarding individual diseases should contact their own physician to obtain medical assistance. The contents in this report are solely the responsibility of the authors and do not necessarily represent the official views of the CDC or of the NCI.

The CBTRUS Scientific Team

Jill Barnholtz-Sloan, Ph.D., CBTRUS Scientific Principal Investigator, Professor, School of Medicine, Case Western Reserve University & Research and Education Institute, University Hospitals Health System, Cleveland, OH

Gino Cioffi, M.P.H., Research Associate, School of Medicine, Case Western Reserve University, Cleveland, OH

Quinn Ostrom, Ph.D., M.P.H., Postdoctoral Associate, Section of Epidemiology and Population Sciences, Department of Medicine, Baylor College of Medicine, Houston, TX

Nirav Patil, M.B.B.S., M.P.H, Senior Biostatistician, Research and Education Institute, University Hospitals Health System, Cleveland, OH

Kristin Waite, Ph.D., Assistant Director, Research Operation, Cleveland Center for Health Outcomes, Case Western Reserve University, Cleveland, OH

The CBTRUS Board of Directors

Carol Kruchko, President & Chief Mission Officer, Central Brain Tumor Registry of the United States, Hinsdale, IL

Steven Brem, M.D., Vice President, Chief of Neurosurgical Oncology, Professor, Department of Neurosurgery, Co-Director, Brain Tumor Center, University of Pennsylvania, Philadelphia, PA

Donald Segal, J.D., Treasurer, Segal McCambridge Singer & Mahoney, Ltd., Chicago, IL

Fred H. Hochberg, M.D., Visiting Scientist, Department of Neurosurgery, University of California at San Diego, San Diego, CA

L. Lloyd Morgan, Patient Advocate, Berkeley, CA

Darell D. Bigner, M.D., Ph.D. (Emeritus Member), Edwin L. Jones, Jr. and Lucille Finch Jones Cancer Research Professor, Department of Pathology, Emeritus Director, The Preston Robert Tisch Brain Tumor Center, Chief, Preuss Laboratory for Brain Tumor Research, Duke, University Medical Center, Durham, NC

Margaret Wrensch, Ph.D., Professor, Neuroepidemiology Division, Department of Neurological Surgery, School of Medicine, University of California-San Francisco, San Francisco, CA

The CBTRUS Board of Advisors

Hoda Anton-Culver, Ph.D., Professor, College of Medicine, University of California-Irvine, Irvine, CA

Melissa Bondy, Ph.D., Professor and Chair, Department of Epidemiology and Population Health, Stanford University School of Medicine, Stanford, CA

Elizabeth B. Claus, M.D., Ph.D., Professor, Departments of Biostatistics and Neurosurgery, Yale University, New Haven, CT, Attending Neurosurgeon, Brigham and Women’s Hospital, Boston, MA

Jennifer Cullen, Ph.D., Associate Professor, School of Medicine, Case Western Reserve University, Cleveland, OH

Faith Davis, Ph.D., Professor, School of Public Health, University of Alberta, Edmonton, Canada

Roberta McKean-Cowdin, Ph.D., Associate Professor, Department of Epidemiology, University of Southern California, Los Angeles, CA

Nancy Stroup, Ph.D., Epidemiologist, retired, GA

John Villano, M.D., Ph.D., Professor, Division of Medical Oncology, University of Kentucky Markey Cancer Center, Lexington, KY

Margaret Wrensch, Ph.D., Professor, Neuroepidemiology Division, Department of Neurological Surgery, School of Medicine, University of California-San Francisco, San Francisco, CA

Funding

CBTRUS is honored to be included among the research awardees of the following organizations which have contributed to the analyses resulting from the CBTRUS database in 2020: the Centers for Disease Control and Prevention (CDC) under Contract No.2016-M-9030, the American Brain Tumor Association, The Sontag Foundation, Novocure, the Musella Foundation, National Brain Tumor Society, the Uncle Kory Foundation, the Pediatric Brain Tumor Foundation, and the Zelda Dorin Tetenbaum Memorial Fund as well as private and in kind donations. QTO is supported by a Research Training Grant from the Cancer Prevention and Research Institute of Texas (CPRIT; RP160097T). Contents are solely the responsibility of the authors and do not necessarily represent the official views of the CDC or of the NCI.

Conflict of interest statement. The authors have no conflicts of interest to report.

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