Abstract
Background:
Brain and other central nervous system (CNS) tumors are a heterogenous collection of tumors, but they are generally reported in local and national cancer statistics as a single, large category. Although the collection of non-malignant brain and other CNS tumors has been mandated since diagnosis year 2004, these tumors are often excluded from standard statistical reports on cancer despite their burden on populations in the United States and Canada. The Central Brain Tumor Registry of the United States (CBTRUS) historical and current histopathological grouping schemes have been developed in collaboration with neuropathologists to capture the diversity of these tumors in clinically relevant categories. The goal of this analysis was to test a new recode variable based on the CBTRUS histopathology grouping prior to releasing the variable for use in the North American Association of Central Cancer Registries (NAACCR) Cancer in North American (CiNA) data sets and by individual cancer registries.
Methods:
The CBTRUS histopathology grouping scheme variable was created and implemented in an evaluation CiNA data set. The accuracy of the variable's categories was evaluated. Counts and incidence rates were calculated using SEER*Stat.
Results:
Overall, 481,650 cases of brain and other CNS tumors meeting the CBTRUS definition were identified for diagnosis years 2015-2019 in the CiNA data set for the US and Canada, making these the sixth-most-common tumor as a group. Of the brain and other CNS tumor cases, approximately 29% were malignant (behavior code /3 in the International Classification of Diseases for Oncology, 3rd edition [ICD-O-3]) while about 71% were nonmalignant (ICD-O-3 behavior code /0 or /1). The overall age-adjusted annual incidence rate (AAAIR) of brain and other CNS tumors was 24.44 per 100,000 (95% CI, 24.37-24.51). The most common histopathologies were meningioma, of which approximately 99% were nonmalignant (AAAIR, 9.09 per 100,000; 95% CI, 9.05-9.13); tumors of the pituitary, of which about 99% were nonmalignant (AAAIR, 4.28 per 100,000; 95% CI, 4.25-4.31); and glioblastoma, of which 100% were malignant behavior (AAAIR, 3.20 per 100,000; 95% CI, 3.18-3.22).
Conclusion:
Brain and other CNS tumors make up an extremely diverse category that contributes substantially to the cancer burden in North America. The CBTRUS histopathology grouping variable provides clinically relevant groupings for analysis of these tumors in the NAACCR CiNA as well as by individual central cancer registry groups. We encourage the use of this variable to support more detailed analysis of this important group of tumors.
Keywords: brain and central nervous system tumors, Central Brain Tumor Registry of the United States, World Health Organization classification of tumors of the central nervous system
Introduction
Brain and other central nervous system (CNS) tumors are a heterogenous group of tumors with more than 100 valid International Classification for Diseases for Oncology, 3rd edition (ICD-O-3) histopathology codes for these sites. While these histopathologies have extremely disparate incidence and outcomes, most cancer statistics report these cases in bulk based on site/topology codes alone and without any stratification by important histopathologic groups. Additionally, approximately 70% of tumors occurring at CNS sites have benign or borderline behavior and may not be included in overall cancer statistics. For most cancers, malignant behavior is the most important predictor of mortality. But for brain and other CNS tumors, location is a primary predictor of significant morbidity. The importance of including all primary brain tumors in cancer reporting was evidenced by Schoenberg et al in the 1970s.1 However, most cancer statistics reporting groups (eg, SEER*Explorer, CiNA Explorer) continue to report only malignant brain and other CNS tumors or report incidence of nonmalignant brain and other CNS tumors separately, thereby underestimating the burden of newly diagnosed primary brain and other CNS tumors.
To address this and to provide clinically relevant statistics on brain and other CNS tumors, the Central Brain Tumor Registry of the United States (CBTRUS) was founded in 1992.2 CBTRUS provided incidence rates from a subset of central cancer registries, resulting in support for the inclusion of "benign" brain tumors by the Centers for Disease Control and Prevention (CDC)'s National Program of Cancer Registries (NPCR).3 As a result of the passage of Public Law 107-260 in 2002 (the Benign Brain Tumor Cancer Registries Amendment Act), benign and borderline brain tumors were collected starting January 1, 2004.4 This law based the collection of brain and other CNS tumors on a site definition rather than a behavior definition as put forth by the currently defunct National Coordinating Council of Cancer Registries and has been agreed upon by members of the cancer surveillance community in concert with representatives from the brain tumor clinical and research communities in Chicago in November 2000 (Consensus Conference 1).5 Since 2009, NPCR has provided data from central cancer registries on brain and other CNS tumors to the CBTRUS, which CBTRUS combines with central cancer registry data from the National Cancer Institute (NCI)'s Surveillance, Epidemiology, and End Results (SEER) Program for its annual reports and publications. Data from 52 central cancer registries (48 from NPCR and 4 from SEER) presented by behavior, histopathology, sex, age, race, Hispanic ethnicity, and geographic location are included in the 2022 CBTRUS statistical report with data from 2015-2019.6
In order to reduce the over 100 histopathologic codes to the clinically appropriate analytic groupings, CBTRUS has worked in collaboration with multiple consulting neuropathologists to develop the CBTRUS histopathologic grouping scheme, which is updated when major changes are made to brain and other CNS tumor classification. In 2016, the WHO Classification of Tumours of the Central Nervous System7 was updated to reflect substantial changes in classification and diagnostic practice that significantly impacted the collection and reporting of CNS tumors. This led to the development of new ICD-O-3 codes, as well as to the development of a new variable (Brain Molecular Markers Site Specific Data Item [SSDI] #3816) by the North American Association of Central Cancer Registries (NAACCR) to fully capture new entities defined in the updated classification.8 These new codes and SSDIs were implemented beginning in diagnosis year 2018. In addition, these changes led to significant updates to both the SEER and CBTRUS histopathologic grouping schemes for tumors of the CNS.9,10
To facilitate broader use of clinically relevant histopa-thology groupings for tumors of the brain and CNS by the broader cancer registry community, CBTRUS has worked with NAACCR to provide the CBTRUS histopathology recode within NAACCR Cancer in North America (CiNA) data sets, including CiNA Public Use.
There are several notable differences in the definition of brain and other CNS tumors between different reporting groups that should be noted when using site-specific recodes. SEER, NAACCR, and NPCR define brain and other CNS tumors in their reporting as tumors located in the brain, meninges, and other parts of the CNS (ICD-O-3 site codes: C70.0-9, C71.0-9, and C72.0-9), with the exclusion of lymphoma and leukemia histopa-thologies (ICD-O-3 codes 9590-9989) occurring at those sites. CBTRUS includes the brain, meninges, other CNS tumors, pituitary, craniopharyngeal duct, and pineal gland (ICD-O-3 site codes C70.0-9, C71.0-9, C72.0-9, C73.3-5), as well as olfactory tumors of the nasal cavity (ICD-O-3 site code C30.0, ICD-O-3 histopathology code 9522-9523 only) and lymphomas and leukemias occurring at brain and CNS sites. The inclusion of tumors of the pituitary, craniopharyngeal duct, and pineal gland as well as primary CNS lymphoma as CNS tumors is in line with the World Health Organization (WHO) classification of CNS tumors, but their inclusion in a site-specific recode is unique to the CBTRUS grouping scheme. CBTRUS also includes all primary brain and other CNS tumors irrespective of behavior. Brain tumors with ICD-O-3 behavior codes /0 (benign) and /1 (borderline) are referred to as nonmalignant brain tumors. Many reports using the term "brain tumor" or "brain cancer" may be restricted to malignant brain tumors only, despite these tumors representing only about 30% of primary brain tumors.6
CBTRUS revised its histopathology groupings to align with the 2016 WHO classification in 2021 while retaining histopathologies that were deemed obsolete for historical comparisons. This process and its potential effect on reporting are detailed at length in Waite, et al.9 The CBTRUS histopathology recode is described in Table 1. CBTRUS also collaborated with SEER to use a new recode that grouped some of the more common histologies into the histopa-thology groupings used in the 2016 WHO classification, and this recode is now available in both publicly available SEER and NAACCR data sets.10 Table 2 provides an overview of classification differences between the SEER and CBTRUS brain and CNS recodes. The objective of this report is to clearly describe the CBTRUS recode and provide guidance for its use by the broader cancer registry community. CBTRUS strongly encourages the use of brain and CNS tumor-specific recodes based on histopathology as opposed to site-based recodes when analyzing brain and CNS data collected by cancer registries for the production of clinically relevant statistics.
Table 1.
Central Brain Tumor Registry of the United States (CBTRUS), 2021 Brain and Other Central Nervous System Tumor Histopathology Groupings (Based on 2016 WHO Classification)
| Histopathology | ICD-O-3 a Histopathology Codesb |
|---|---|
| Diffuse astrocytic and oligodendroglial tumors | |
| Diffuse astrocytoma* | 9381, 9400, 9410, 9411, 9420, 9442/1 |
| Anaplastic astrocytoma* | 9401 |
| Glioblastoma* | 9440, 9441, 9442/3, 9445c |
| Oligodendroglioma* | 9450 |
| Anaplastic oligodendroglioma* | 9451, 9460 |
| Oligoastrocytic tumors* | 9382 |
| Other astrocytic Tumors | |
| Pilocytic astrocytoma* | 9421, 9425c |
| Unique astrocytoma variants* | 9384, 9424, 9431c |
| Ependymal tumors* | 9383, 9391 (excluding site C75.1 for behavior /1), 9392-9394, 9396c |
| Other gliomas | |
| Glioma malignant, NOS* | 9380, 9385c |
| Other neuroepithelial tumors* | 9423, 9430, 9444 |
| Neuronal and mixed neuronal-glial tumors* | 8680, 8681, 8690, 8693, 9412, 9413, 9490, 9492 (excluding site C75.1), 9493, 9505, 9506, 9509c, 9522 (site C30.0 only), 9523 (site C30.0 only) |
| Choroid plexus tumors | 9390 |
| Tumors of the pineal region | 9360, 9361, 9362, 9395c |
| Embryonal tumors | 8963, 9364, 9470-9478c, 9480, 9500, 9501/3, 9502/3, 9508 |
| Medulloblastoma | 9470-9472,9474-9478 |
| Atypical teratoid rhabdoid tumor | 9508 |
| Other embryonal tumors | 8963, 9364, 9473, 9480, 9500, 9501, 9502 |
| Tumors of cranial and paraspinal nerves | |
| Nerve sheath tumors | 9540, 9541, 9550, 9560, 9561, 9570, 9571 |
| Other tumors of cranial and paraspinal nerves | 9562, 9563 |
| Tumors of meninges | |
| Meningioma | 9530-9535, 9537-9539 |
| Mesenchymal tumors | 8324, 8710, 8711, 8800-8806, 8810, 8811, 8815, 8821, 8824, 8825, 8830, 8831, 8835, 8836, 8840, 8850-8854, 8857, 8861, 8870, 8880, 8890, 8897, 8900-8902, 8910, 8912, 8920, 8921, 8935, 8990, 9040, 9120, 9125, 9130, 9131, 9133, 9136, 9150, 9161, 9170, 9180, 9210, 9220, 9231, 9240, 9241, 9243, 9260, 9370-9373 |
| Primary melanocytic lesions | 8720, 8728, 8770 |
| Other neoplasms related to the meninges | None |
| Lymphomas and hematopoietic neoplasms | |
| Lymphoma | 9590, 9591, 9596, 9650-9655, 9659, 9661-9665, 9667, 9670, 9671, 9673, 9675, 9680, 9684, 9687, 9688, 9690, 9691, 9695, 9698, 9699, 9701, 9702, 9705, 9712, 9714, 9715, 9719, 9724, 9727-9729, 9735, 9737, 9738, 9750, 9751, 9755, 9756, 981 1-9819, 9823, 9826, 9827, 9831, 9832, 9837, 9861, 9866, 9930, 9965, 9966, 9967, 9970, 9971, 9975 |
| Other hematopoietic neoplasms | 9731, 9733, 9734, 9740, 9741, 9749, 9752-9754, 9757-9758, 9759, 9760, 9766, 9860, |
| Germ cell tumors | 8440, 9060, 9061, 9064, 9065, 9070-9072, 9080-9083, 9084/3, 9085, 9100, 9101 |
| Tumors of sellar region | |
| Tumors of the pituitary | 8040 (site C75.1 only), 8140 (site C75.1 only), 8146 (site C75.1 only), 8246, 8260 (site C75.1 only), 8270-8272, 8280, 8281, 8290, 8300, 8310, 8323, 9391/1 (site C75.1 only), 9432c (site C75.1 only), 9492 (site C75.1 only), 9580, 9582 |
| Craniopharyngioma | 9350-9352 |
| Unclassified tumors | |
| Hemangioma | 9121-9123, 9133, 9140 |
| Neoplasm, unspecified | 8000-8005, 8010, 8020, 8021 |
| All other | 8320, 8452, 8713, 8896, 8963, 8980, 9084/0, 9173, 9363, 9503 |
NOS, not otherwise specified; WHO, World Health Organization. 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 histopathology codes included in each group: https://www.cbtrus.org. c Added starting with diagnosis year 2018.
All or some of this histopathology is included in the CBTRUS definition of gliomas, including ICD-O-3 histopathology codes 9380-9384 and 9391-9460.
Table 2.
Frequency of Cases Assigned to Categories in the CBTRUS and SEER Brain and CNS Histopathologic Recode Schemes, 2015-2019 NAACCR CiNA
Methods
The NAACCR CiNA data set used for this analysis includes data from 67 central cancer registries: 56 from the United States (49 states [excluding Nevada due to data quality issues], the District of Columbia, and Puerto Rico, and 5 regions [California, excluding the Greater Bay area and Los Angeles, plus 4 metropolitan areas]) and 11 from Canada (9 provinces and 2 territories).11 A special evaluation version of a CiNA Public Use data set12 was used, which included a recode variable based on a standard SEER*Stat variable, Site Recode ICD-O-3/WHO 2008, and the ICD-O-3 histology codes. The recode variable was based on the Site Recode ICD-O-3/WHO 2008 equal to Brain and Other Nervous System (ICD-O-3 codes C70.0-72.9), and histology was aligned with the CBTRUS groupings. For the purposes of this evaluation, the CBTRUS brain and CNS site definition was used (ICD-O-3 site codes C70.0-9, C71.0-9, C72.0-9, C73.3-5, as well as C30.0) for ICD-O-3 histopathology code 9522-9523 only and behavior codes of /0, /1, and /3.
CNS, central nervous system; NAACCR CiNA, North American Association of Central Cancer Registries Cancer in North America; NOS, not otherwise specified. a Rates are per 100,000 and are age-adjusted to the 2000 US standard population. b Percentages may not add up to 100% due to rounding.
Counts, average annual age-adjusted incidence rates (AAAIR) per 100,000 population, and 95% CIs by CBTRUS histopathology groupings, behavior, and sex were generated for diagnosis years 2015-2019 for all registries included in the special NAACCR CiNA data set using SEER*Stat 8.4.0.13
Results
Overall, 481,650 cases (96,330 cases annually on average) of brain and other CNS tumors meeting the CBTRUS definition were identified in the 2015-2019 NAACCR CiNA data set, including both US and Canadian central cancer registries (Table 3). This is about 25% higher than when the SEER brain and CNS site recode definition is used to identify tumors of the brain and CNS (383,949 total cases for the 5-year period, for an annual average of 76,790 cases), and about 282% higher than when cases are limited to those with malignant behavior only ICD-O-3 behavior code of /3 (125,961 total cases for the 5-year period, for an annual average of 25,192 cases).
Table 3.
Five-Year Total, Annual Average Total a, and Average Annual Age-Adjusted Incidence Rates b with 95% Confidence Intervals for All Brain and Other Central Nervous System Tumors by Major Histopathology Groupings, Histopathology, Behavior, and Sex, 2015-2019 NAACCR CiNA
| Histopathology | 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) | |
| Diffuse astrocytic and oligodendroglial tumors | 91,847 | 18,369 | 4.50 (4.47-4.53) | 52,901 | 10,580 | 5.49 (5.44-5.54) | 38,946 | 7,789 | 3.64 (3.60-3.68) |
| Diffuse astrocytoma | 9,687 | 1,937 | 0.52 (0.51-0.53) | 5,456 | 1,091 | 0.60 (0.59-0.62) | 4,231 | 846 | 0.45 (0.43-0.46) |
| Anaplastic astrocytoma | 7,526 | 1,505 | 0.40 (0.39-0.41) | 4,120 | 824 | 0.45 (0.44-0.47) | 3,406 | 681 | 0.35 (0.34-0.36) |
| Glioblastoma | 67,827 | 13,565 | 3.20 (3.18-3.22) | 39,549 | 7,910 | 4.00 (3.96-4.04) | 28,278 | 5,656 | 2.50 (2.48-2.54) |
| Oligodendroglioma | 4,048 | 810 | 0.23 (0.23-0.24) | 2,247 | 449 | 0.26 (0.25-0.27) | 1,801 | 360 | 0.20 (0.20-0.21) |
| Anaplastic oligodendroglioma | 2,164 | 433 | 0.12 (0.11-0.12) | 1,209 | 242 | 0.14 (0.13-0.14) | 955 | 191 | 0.10 (0.09-0.11) |
| Oligoastrocytic tumors | 595 | 119 | 0.03 (0.03-0.04) | 320 | 64 | 0.04 (0.03-0.04) | 275 | 55 | 0.03 (0.03-0.03) |
| Other astrocytic tumors | 6,701 | 1,340 | 0.42 (0.41-0.43) | 3,545 | 709 | 0.44 (0.42-0.45) | 3,156 | 631 | 0.40 (0.39-0.42) |
| Pilocytic astrocytoma | 5,706 | 1,141 | 0.36 (0.35-0.37) | 3,003 | 601 | 0.37 (0.36-0.39) | 2,703 | 541 | 0.35 (0.33-0.36) |
| Unique astrocytoma variants | 995 | 199 | 0.06 (0.06-0.06) | 542 | 108 | 0.07 (0.06-0.07) | 453 | 91 | 0.06 (0.05-0.06) |
| Malignant | 573 | 115 | 0.03 (0.03-0.04) | 296 | 59 | 0.04 (0.03-0.04) | 277 | 55 | 0.03 (0.03-0.04) |
| Nonmalignant | 422 | 84 | 0.03 (0.02-0.03) | 246 | 49 | 0.03 (0.03-0.03) | 176 | 35 | 0.02 (0.02-0.03) |
| Ependymal tumors | 7,460 | 1,492 | 0.42 (0.41-0.43) | 4,302 | 860 | 0.49 (0.47-0.50) | 3,158 | 632 | 0.35 (0.34-0.36) |
| Malignant | 4,110 | 822 | 0.23 (0.23-0.24) | 2,250 | 450 | 0.26 (0.25-0.27) | 1,860 | 372 | 0.21 (0.20-0.22) |
| Nonmalignant | 3,350 | 670 | 0.18 (0.18-0.19) | 2,052 | 410 | 0.23 (0.22-0.24) | 1,298 | 260 | 0.14 (0.13-0.15) |
| Other gliomas | 9,672 | 1,934 | 0.55 (0.54-0.56) | 4,909 | 982 | 0.57 (0.56-0.59) | 4,763 | 953 | 0.53 (0.51-0.54) |
| Glioma malignant, NOS | 9,561 | 1,912 | 0.54 (0.53-0.55) | 4,865 | 973 | 0.57 (0.55-0.58) | 4,696 | 939 | 0.52 (0.50-0.54) |
| Other neuroepithelial tumors | 111 | 22 | 0.01 (0.01-0.01) | 44 | 9 | 0.01 (0.00-0.01) | 67 | 13 | 0.01 (0.01-0.01) |
| Neuronal and mixed neuronal-glial tumors | 5,805 | 1,161 | 0.34 (0.33-0.35) | 3,154 | 631 | 0.37 (0.36-0.38) | 2,651 | 530 | 0.31 (0.30-0.32) |
| Malignant | 1,066 | 213 | 0.06 (0.05-0.06) | 588 | 118 | 0.06 (0.06-0.07) | 478 | 96 | 0.05 (0.05-0.05) |
| Nonmalignant | 4,739 | 948 | 0.28 (0.28-0.29) | 2,566 | 513 | 0.31 (0.29-0.32) | 2,173 | 435 | 0.26 (0.25-0.27) |
| Choroid plexus tumors | 880 | 176 | 0.05 (0.05-0.06) | 441 | 88 | 0.05 (0.05-0.06) | 439 | 88 | 0.05 (0.05-0.06) |
| Malignant | 125 | 25 | 0.01 (0.01-0.01) | 74 | 15 | 0.01 (0.01-0.01) | 51 | 10 | 0.01 (0.00-0.01) |
| Nonmalignant | 755 | 151 | 0.04 (0.04-0.05) | 367 | 73 | 0.04 (0.04-0.05) | 388 | 78 | 0.04 (0.04-0.05) |
| Tumors of the pineal region | d | 167 | 0.05 (0.04-0.05) | 352 | 70 | 0.04 (0.04-0.05) | 484 | 97 | 0.06 (0.05-0.06) |
| Malignant | 508 | 102 | 0.03 (0.03-0.03) | 243 | 49 | 0.03 (0.02-0.03) | 265 | 53 | 0.03 (0.03-0.04) |
| Nonmalignant | 328 | 66 | 0.02 (0.02-0.02) | 109 | 22 | 0.01 (0.01-0.01) | 219 | 44 | 0.02 (0.02-0.03) |
| Embryonal tumors | 3,453 | 691 | 0.22 (0.21-0.23) | 2,086 | 417 | 0.26 (0.25-0.27) | 1,367 | 273 | 0.18 (0.17-0.19) |
| Tumors of cranial and paraspinal nerves | 39,749 | 7,950 | 2.02 (2.00-2.04) | 19,099 | 3,820 | 2.02 (1.99-2.05) | 20,650 | 4,130 | 2.03 (2.00-2.06) |
| Nerve sheath tumors | 39,714 | 7,943 | 2.02 (2.00-2.04) | 19,078 | 3,816 | 2.01 (1.99-2.04) | 20,636 | 4,127 | 2.03 (2.00-2.05) |
| Malignant | 233 | 47 | 0.01 (0.01-0.01) | 118 | 24 | 0.01 (0.01-0.02) | 115 | 23 | 0.01 (0.01-0.01) |
| Nonmalignant | 39,481 | 7,896 | 2.00 (1.98-2.02) | 18,960 | 3,792 | 2.00 (1.97-2.03) | 20,521 | 4,104 | 2.01 (1.99-2.04) |
| Other tumors of cranial and paraspinal nerves | 35 | 7 | 0.00 (0.00-0.00) | 21 | 4 | 0.00 (0.00-0.00) | 14 | 3 | 0.00 (0.00-0.00) |
| Tumors of meninges | 193,788 | 38,758 | 9.48 (9.44-9.52) | 54,414 | 10,883 | 5.80 (5.76-5.86) | 139,374 | 27,875 | 12.76 (12.69-12.83) |
| Meningioma | 186,524 | 37,305 | 9.09 (9.05-9.13) | 50,719 | 10,144 | 5.40 (5.35-5.45) | 135,805 | 27,161 | 12.38 (12.31-12.45) |
| Malignant | 1,768 | 354 | 0.09 (0.08-0.09) | 823 | 165 | 0.09 (0.08-0.09) | 945 | 189 | 0.09 (0.08-0.09) |
| Nonmalignant | 184,756 | 36,951 | 9.00 (8.96-9.04) | 49,896 | 9,979 | 5.31 (5.26-5.36) | 134,860 | 26,972 | 12.29 (12.22-12.36) |
| Mesenchymal tumors | 7,103 | 1,421 | 0.38 (0.37-0.39) | 3,603 | 721 | 0.40 (0.38-0.41) | 3,500 | 700 | 0.37 (0.36-0.39) |
| Malignant | 851 | 170 | 0.05 (0.04-0.05) | 452 | 90 | 0.05 (0.05-0.06) | 399 | 80 | 0.04 (0.04-0.05) |
| Nonmalignant | 6,252 | 1,250 | 0.34 (0.33-0.35) | 3,151 | 630 | 0.35 (0.33-0.36) | 3,101 | 620 | 0.33 (0.32-0.34) |
| Primary melanocytic lesions | 161 | 32 | 0.01 (0.01-0.01) | 92 | 18 | 0.01 0.01-0.01) | 69 | 14 | 0.01 (0.01-0.01) |
| Lymphomas and hematopoietic neoplasms | 9,259 | 1,852 | 0.44 (0.43-0.45) | 4,764 | 953 | 0.49 (0.48-0.51) | 4,495 | 899 | 0.40 (0.39-0.41) |
| Lymphoma | 9,210 | 1,842 | 0.44 (0.43-0.45) | 4,736 | 947 | 0.49 (0.48-0.50) | 4,474 | 895 | 0.40 (0.39-0.41) |
| Other hematopoietic neoplasms | 49 | 10 | 0.00 (0.00-0.00) | 28 | 6 | 0.00 (0.00-0.00) | 21 | 4 | 0.00 (0.00-0.00) |
| Germ cell tumors | 1,769 | 354 | 0.11 (0.10-0.11) | 1,226 | 245 | 0.15 (0.14-0.16) | 543 | 109 | 0.07 (0.06-0.07) |
| Malignant | 1,229 | 246 | 0.08 (0.07-0.08) | 938 | 188 | 0.11 (0.11-0.12) | 291 | 58 | 0.04 (0.03-0.04) |
| Nonmalignant | 540 | 108 | 0.03 (0.03-0.04) | 288 | 58 | 0.03 (0.03-0.04) | 252 | 50 | 0.03 (0.03-0.03) |
| Tumors of sellar region | 83,392 | 16,678 | 4.47 (4.44-4.50) | 37,348 | 7,470 | 4.01 (3.97-4.05) | 46,044 | 9,209 | 5.01 (4.96-5.06) |
| Tumors of the pituitary | 80,004 | 16,001 | 4.28 (4.25-4.31) | 35,593 | 7,119 | 3.81 (3.77-3.86) | 44,411 | 8,882 | 4.83 (4.78-4.88) |
| Malignant | 118 | 24 | 0.01 (0.00-0.01) | ||||||
| Nonmalignant | 79,886 | 15,977 | 4.28 (4.25-4.31) | ||||||
| Craniopharyngioma | 3,388 | 678 | 0.19 (0.18-0.19) | 1,755 | 351 | 0.20 (0.19-0.21) | 1,633 | 327 | 0.18 (0.17-0.19) |
| Unclassified tumors | 26,973 | 5,395 | 1.37 (1.35-1.39) | 12,213 | 2,443 | 1.36 (1.34-1.39) | 14,760 | 2,952 | 1.39 (1.36-1.41) |
| Hemangioma | 4,377 | 875 | 0.24 (0.23-0.24) | 2,047 | 409 | 0.23 (0.22-0.24) | 2,330 | 466 | 0.25 (0.24-0.26) |
| Neoplasm, unspecified | 22,240 | 4,448 | 1.11 (1.10-1.13) | 9,971 | 1,994 | 1.11 (1.09-1.13) | 12,269 | 2,454 | 1.12 (1.10-1.14) |
| Malignant | 7,584 | 1,517 | 0.37 (0.36-0.38) | 3,693 | 739 | 0.41 (0.40-0.43) | 3,891 | 778 | 0.33 (0.32-0.34) |
| Nonmalignant | 14,656 | 2,931 | 0.74 (0.73-0.76) | 6,278 | 1,256 | 0.70 (0.68-0.71) | 8,378 | 1,676 | 0.79 (0.77-0.81) |
| All other | 206 | 41 | 0.01 (0.01-0.01) | 126 | 25 | 0.01 (0.01-0.02) | 80 | 16 | 0.01 (0.01-0.01) |
| Brain/CNS not categorized c | 150 | 30 | 0.01 (0.01-0.01) | 69 | 14 | 0.01 (0.01-0.01) | 81 | 16 | 0.01 (0.01-0.01) |
| Total d | 481,650 | 96,330 | 24.44 (24.37-24.51) | 200,781 | 40,156 | 21.55 (21.45-21.64) | 280,869 | 56,174 | 27.16 (27.06-27.27) |
| Malignant | 137,617 | 27,523 | 7.00 (6.96-7.04) | 76,982 | 15,396 | 8.23 (8.18-8.29) | 60,635 | 12,127 | 5.90 (5.85-5.95) |
| Nonmalignant | 344,033 | 68,807 | 17.44 (17.38-17.50) | 123,799 | 24,760 | 13.31 (13.24-13.39) | 220,234 | 44,047 | 21.26 (21.17-21.35) |
ICD-O-3, International Classification of Diseases for Oncology, 3rd edition; NOS, not otherwise specified.
Annual average cases are calculated by dividing the five-year total by five
Rates are per 100,000 and are age-adjusted to the 2000 US standard population.
Includes ICD-O-3 codes fitting site criteria that are not included in CBTRUS histopathology classification
Refers to all brain tumors including histopathologies 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.
In comparison to other common cancers in the CiNA data set, brain and other CNS tumors are the sixth most commonly occurring tumor when using the CBTRUS definition (Figure 1, Panel A). Of the 481,650 cases of primary brain tumors, 137,617 (28.6%) were malignant (ICD-O-3 behavior code /3) while 344,033 (71.4%) were nonmalignant (ICD-O-3 behavior code /0 or /1) (Figure 1, Panel B). The overall AAAIR of brain and other CNS tumors was 24.44 per 100,000 (95% CI, 24.37-24.51). The incidence of malignant tumors only was 7.00 per 100,000 (95% CI, 6.96-7.04), while the incidence of nonmalignant tumors was 17.44 per 100,000 (95% CI, 17.38-17.50).
Figure 1.
(A) Average Annual Age-Adjusted Incidence Rates (AAAIRs) with 95% CIs of All Primary Brain and Other Central Nervous System Tumors in Comparison to Top 8 Highest Incidence Cancers and (B) Distribution of All Primary Brain and Other Central Nervous System Tumors by Behavior and Most Common Histopathologies, 2015-2019 NAACCR CiNA
Brain and other CNS tumors occurred more frequently in females (AAAIR, 27.16 per 100,000; 95% CI, 27. 06-27.27) than in males (AAAIR, 21.55 per 100,000; 95% CI, 21.4521.64). For malignant tumors only, incidence was higher in males than females (male AAAIR, 8.23 per 100,000; 95% CI, 8.18-8.29 compared to female AAAIR, 5.90 per 100,000; 95% CI, 5.85-5.95). The opposite was true in nonmalig-nant tumors, where incidence was nearly twice as high in females (female AAAIR, 21.26 per 100,000; 95% CI, 21.17-21.35) than in males (male AAAIR, 13.31 per 100,000; 95% CI, 13.24-13.39)
The most frequently occurring histopathology was meningioma (AAAIR, 9.09 per 100,000; 95% CI, 9.05-9.13), of which about 99% are nonmalignant (Table 2). Meningioma occurred more than twice as frequently in females (female AAAIR, 12.38 per 100,000; 95% CI, 12.31-12.45) than in males (male AAAIR, 5.40 per 100,000; 95% CI, 5.35-5.45). The second most frequently occurring histopathology was tumors of the pituitary (AAAIR, 4.28 per 100,000; 95% CI, 4.25-4.31), followed by glioblastoma (AAAIR, 3.20 per 100,000; 95% CI, 3.18-3.22). The predominantly nonmalig-nant tumors of the pituitary occurred at much higher rates in females than in males (female AAAIR, 4.83 per 100,000; 95% CI, 4.78-4.88 as compared to male AAAIR, 3.81 per 100,000; 95% CI, 3.77-3.86). Glioblastoma occurred more commonly in males than in females (male AAAIR, 4.00 per 100,000; 95% CI, 3.96-4.04 as compared to female AAAIR, 2.50 per 100,000; 95% CI, 2.48-2.54).
Incidence estimates by age group are shown in Table 4. Overall, brain and CNS tumors are most common in those older than 40 years (>40 years AAAIR, 44.11 per 100,000; 95% CI, 43.97-44.25, as compared to 0-14 years AAAIR, 5.81 per 100,000; 95% CI, 5.73-5.90, and 15-39 years AAAIR, 11.84 per 100,000; 95% CI, 11.75-11.93). The most frequently occurring histopathology in children aged 0-14 years was pilocytic astrocytoma (AAAIR, 1.11 per 100,000; 95% CI, 1.07-1.15), while in adolescents and young adults aged 15-39 years, it was tumors of the pituitary (AAAIR, 4.03 per 100,000; 95% CI, 3.98-4.08). In older adults (>40 years), meningioma was reported as the most frequently occurring histopathology (AAAIR, 19.49 per 100,000; 95% CI, 19.40-19.59).
Table 4.
Five-Year Total, Annual Average Total,a and Average Annual Age-Adjusted Incidence Rates b with 95% CIs for All Brain and Other Central Nervous System Tumors by Major Histopathology Groupings, Histopathology, Behavior, and Age Group, 2015-2019 NAACCR CiNA
| Histopathology | 0-14 y | 15-39 y | >40 y | ||||||
|---|---|---|---|---|---|---|---|---|---|
| 5-year total | Annual average | 95% CI | 5-year total | Annual average | 95% CI | 5-year total | Annual average | 95% CI | |
| Diffuse astrocytic and oligodendroglial tumors | 1,394 | 279 | 0.46 (0.43-0.48) | 10,737 | 2,147 | 1.84 (1.81-1.88) | 79,598 | 15,920 | 8.71 (8.65-8.78) |
| Diffuse astrocytoma | 630 | 126 | 0.21 (0.19-0.22) | 2,901 | 580 | 0.49 (0.47-0.51) | 6,113 | 1,223 | 0.70 (0.68-0.72) |
| Anaplastic astrocytoma | 222 | 44 | 0.07 (0.06-0.08) | 2,189 | 438 | 0.37 (0.36-0.39) | 5,097 | 1,019 | 0.59 (0.57-0.60) |
| Glioblastoma | 448 | 90 | 0.15 (0.13-0.16) | 3,282 | 656 | 0.57 (0.55-0.59) | 64,045 | 12,809 | 6.89 (6.83-6.94) |
| Oligodendroglioma | 62 | 12 | 0.02 (0.02-0.03) | 1,578 | 316 | 0.27 (0.26-0.29) | 2,404 | 481 | 0.31 (0.29-0.32) |
| Anaplastic oligodendroglioma | - | - | - | - | - | - | 1,574 | 315 | 0.19 (0.18-0.20) |
| Oligoastrocytic tumors | - | - | - | - | - | - | 365 | 73 | 0.04 (0.04-0.05) |
| Other astrocytic tumors | 3,760 | 752 | 1.23 (1.19-1.27) | 1,982 | 396 | 0.34 (0.32-0.35) | 821 | 164 | 0.10 (0.10-0.11) |
| Pilocytic astrocytoma | 3,395 | 679 | 1.11 (1.07-1.15) | 1,566 | 313 | 0.27 (0.25-0.28) | 637 | 127 | 0.08 (0.07-0.09) |
| Unique astrocytoma variants | 365 | 73 | 0.12 (0.11-0.13) | 416 | 83 | 0.07 (0.06-0.08) | 184 | 37 | 0.02 (0.02-0.03) |
| Malignant | - | - | - | 281 | 56 | 0.05 (0.04-0.05) | - | - | - |
| Nonmalignant | - | - | - | 135 | 27 | 0.02 (0.02-0.03) | - | - | - |
| Ependymal tumors | 931 | 186 | 0.30 (0.28-0.32) | 2,069 | 414 | 0.36 (0.34-0.37) | 4,396 | 879 | 0.52 (0.51-0.54) |
| Malignant | 812 | 162 | 0.26 (0.25-0.28) | 1,060 | 212 | 0.18 (0.17-0.19) | 2,182 | 436 | 0.26 (0.25-0.27) |
| Nonmalignant | 119 | 24 | 0.04 (0.03-0.05) | 1,009 | 202 | 0.17 (0.16-0.18) | 2,214 | 443 | 0.26 (0.25-0.27) |
| Other gliomas | 2,761 | 552 | 0.90 (0.87-0.94) | 2,085 | 417 | 0.35 (0.34-0.37) | 4,730 | 946 | 0.54 (0.53-0.56) |
| Glioma malignant, NOS | 2,739 | 548 | 0.90 (0.86-0.93) | 2,045 | 409 | 0.35 (0.33-0.36) | 4,682 | 936 | 0.54 (0.52-0.55) |
| Other neuroepithelial tumors | 22 | 4 | 0.01 (0.00-0.01) | 40 | 8 | 0.01 (0.00-0.01) | 48 | 10 | 0.01 (0.00-0.01) |
| Neuronal and mixed neuronal-glial tumors | 1,391 | 278 | 0.46 (0.43-0.48) | 2,353 | 471 | 0.40 (0.38-0.41) | 1,968 | 394 | 0.24 (0.23-0.25) |
| Malignant | 86 | 17 | 0.03 (0.02-0.03) | 210 | 42 | 0.04 (0.03-0.04) | 758 | 152 | 0.09 (0.08-0.09) |
| Nonmalignant | 1,305 | 261 | 0.43 (0.40-0.45) | 2,143 | 429 | 0.36 (0.35-0.38) | 1,210 | 242 | 0.15 (0.14-0.16) |
| Choroid plexus tumors | 252 | 50 | 0.08 (0.07-0.09) | 220 | 44 | 0.04 (0.03-0.04) | 282 | 56 | 0.03 (0.03-0.04) |
| Malignant | 62 | 12 | 0.02 (0.02-0.03) | - | - | - | - | - | - |
| Nonmalignant | 190 | 38 | 0.06 (0.05-0.07) | - | - | - | - | - | - |
| Tumors of the pineal region | 150 | 30 | 0.05 (0.04-0.06) | 315 | 63 | 0.05 (0.05-0.06) | 363 | 73 | 0.04 (0.04-0.05) |
| Malignant | 131 | 26 | 0.04 (0.04-0.05) | 191 | 38 | 0.03 (0.03-0.04) | 181 | 36 | 0.02 (0.02-0.03) |
| Nonmalignant | 19 | 4 | 0.01 (0.00-0.01) | 124 | 25 | 0.02 (0.02-0.03) | 182 | 36 | 0.02 (0.02-0.03) |
| Embryonal tumors | 2,070 | 414 | 0.68 (0.65-0.71) | 820 | 164 | 0.14 (0.13-0.15) | 303 | 61 | 0.04 (0.03-0.04) |
| Tumors of cranial and paraspinal nerves | 713 | 143 | 0.23 (0.22-0.25) | 6,024 | 1,205 | 1.05 (1.02-1.07) | 32,985 | 6,597 | 3.71 (3.67-3.75) |
| Nerve sheath tumors | 713 | 143 | 0.23 (0.22-0.25) | 6,017 | 1,203 | 1.05 (1.02-1.07) | 32,957 | 6,591 | 3.71 (3.67-3.75) |
| Malignant | - | - | - | - | - | - | 164 | 33 | 0.02 (0.02-0.02) |
| Nonmalignant | - | - | - | - | - | - | 32,793 | 6,559 | 3.69 (3.65-3.73) |
| Other tumors of cranial and paraspinal nerves | - | - | - | - | - | - | 28 | 6 | 0.00 (0.00-0.00) |
| Tumors of meninges | 756 | 151 | 0.25 (0.23-0.27) | 12,626 | 2,525 | 2.24 (2.20-2.28) | 180,209 | 36,042 | 20.04 (19.94-20.13) |
| Meningioma | 317 | 63 | 0.10 (0.09-0.12) | 10,699 | 2,140 | 1.91 (1.88-1.95) | 175,491 | 35,098 | 19.49 (19.40-19.59) |
| Malignant | - | - | - | - | - | - | 1,626 | 325 | 0.18 (0.17-0.19) |
| Nonmalignant | - | - | - | - | - | - | 173,865 | 34,773 | 19.31 (19.22-19.41) |
| Mesenchymal tumors | 429 | 86 | 0.14 (0.13-0.15) | 1,898 | 380 | 0.32 (0.31-0.34) | 4,596 | 919 | 0.53 (0.52-0.55) |
| Malignant | 72 | 14 | 0.02 (0.02-0.03) | 184 | 37 | 0.03 (0.03-0.04) | 590 | 118 | 0.07 (0.06-0.07) |
| Nonmalignant | 357 | 71 | 0.12 (0.10-0.13) | 1,714 | 343 | 0.29 (0.28-0.31) | 4,006 | 801 | 0.46 (0.45-0.48) |
| Primary melanocytic lesions | 10 | 2 | 0.00 (0.00-0.01) | 29 | 6 | 0.00 (0.00-0.01) | 122 | 24 | 0.01 (0.01-0.02) |
| Lymphomas and hematopoietic neoplasms | 87 | 17 | 0.03 (0.02-0.04) | 580 | 116 | 0.10 (0.09-0.11) | 8,588 | 1,718 | 0.94 (0.92-0.96) |
| Lymphoma | - | - | - | - | - | - | 8,545 | 1,709 | 0.93 (0.91-0.95) |
| Other hematopoietic neoplasms | - | - | - | - | - | - | 43 | 9 | 0.00 (0.00-0.01) |
| Germ cell tumors | 680 | 136 | 0.22 (0.21-0.24) | 792 | 158 | 0.13 (0.12-0.14) | 215 | 43 | 0.03 (0.02-0.03) |
| Malignant | 513 | 103 | 0.17 (0.15-0.18) | 635 | 127 | 0.11 (0.10-0.11) | - | - | - |
| Nonmalignant | 167 | 33 | 0.05 (0.05-0.06) | 157 | 31 | 0.03 (0.02-0.03) | - | - | - |
| Tumors of sellar region | 1,811 | 362 | 0.59 (0.57-0.62) | 24,408 | 4,882 | 4.16 (4.11-4.21) | 57,157 | 11,431 | 6.68 (6.62-6.73) |
| Tumors of the pituitary | 1,110 | 222 | 0.36 (0.34-0.39) | 23,663 | 4,733 | 4.03 (3.98-4.08) | 55,222 | 11,044 | 6.45 (6.40-6.51) |
| Malignant | - | - | - | - | - | - | 98 | 20 | 0.01 (0.01-0.01) |
| Nonmalignant | - | - | - | - | - | - | 55,124 | 11,025 | 6.44 (6.39-6.50) |
| Craniopharyngioma | 701 | 140 | 0.23 (0.21-0.25) | 745 | 149 | 0.13 (0.12-0.14) | 1,935 | 387 | 0.22 (0.21-0.23) |
| Unclassified tumors | 1,014 | 203 | 0.33 (0.31-0.35) | 3,748 | 750 | 0.64 (0.62-0.66) | 22,096 | 4,419 | 2.48 (2.45-2.51) |
| Hemangioma | 278 | 56 | 0.09 (0.08-0.10) | 1,207 | 241 | 0.21 (0.19-0.22) | 2,876 | 575 | 0.34 (0.32-0.35) |
| Neoplasm, unspecified | 698 | 140 | 0.23 (0.21-0.25) | 2,491 | 498 | 0.43 (0.41-0.45) | 18,970 | 3,794 | 2.11 (2.08-2.15) |
| Malignant | 146 | 29 | 0.05 (0.04-0.06) | 380 | 76 | 0.07 (0.06-0.07) | 7,029 | 1,406 | 0.77 (0.76-0.79) |
| Nonmalignant | 552 | H0 | 0.18 (0.17-0.20) | 2,111 | 422 | 0.36 (0.35-0.38) | 11,941 | 2,388 | 1.34 (1.31-1.36) |
| All other | 28 | 6 | 0.01 (0.01-0.01) | 23 | 5 | 0.00 (0.00-0.01) | 142 | 28 | 0.02 (0.01-0.02) |
| Brain/CNS not categorized c | - | - | - | - | - | - | 108 | 22 | 0.01 (0.01-0.01) |
| Total d | 17,773 | 3,555 | 5.81 (5.73-5.90) | 68,780 | 13,756 | 11.84 (1 1.75-11.93) | 393,753 | 78,751 | 44.11 (43.97-44.25) |
| Malignant | 11,421 | 2,284 | 3.73 (3.67-3.80) | 18,841 | 3,768 | 3.22 (3.18-3.27) | 106,578 | 21,316 | 11.74 (11.67-11.82) |
| Nonmalignant | 6,352 | 1,270 | 2.08 (2.03-2.13) | 49,939 | 9,988 | 8.62 (8.54-8.69) | 287,175 | 57,435 | 32.36 (32.24-32.49) |
CBTRUS, Central Brain Tumor Registry of the United States; CNS, central nervous system; NAACCR CiNA, North American Association of Central Cancer Registries Cancer in North America; NOS, not otherwise specified. Annual average cases are calculated by dividing the 5-year total by 5. 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.
Annual average cases are calculated by dividing the five-year total by five
Rates are per 100,000 and are age-adjusted to the 2000 US standard population.
Includes ICD-O-3 codes fitting site criteria that are not included in CBTRUS histopathology classification
Refers to all brain tumors including histopathologies not presented in this table.
Discussion
CBTRUS produces annual statistical reports using this CBTRUS histopathology grouping scheme, the most recent of which was based on 445,792 cases diagnosed from 2015-2019.6 This includes data from 51 central cancer registries in the United States only (50 states and Washington DC; about 100% of the United States with the exception of Nevada from the years 2018-2019), and is therefore smaller than the CiNA data, which includes Canadian registries. The results of this initial evaluation show that, while case counts vary due to differences in included central cancer registries, the overall incidence patterns for primary brain and other CNS tumors are the same within the CBTRUS analytic data set and the NAACCR CiNA data set. Observed overall patterns, incidence by sex, and incidence by age are very similar to those estimated using the CBTRUS data set. Variations may occur when using the recode variable in data sets that may include lower proportions of nonma-lignant tumors or variations in the proportion of cases abstracted from radiographic imaging, which is associated with increased detection (and therefore higher incidence) of nonmalignant brain and other CNS tumors. Care should be taken when generating and interpreting statistics for these tumors, particularly concerning the underlying site definition used, as these vary by major reporting organizations.
Incidence rates for brain and other CNS tumors vary significantly by histopathology, sex, age, and other demographic factors, including race/ethnicity and geography. The CBTRUS variable provides valuable information for comparing the burden of brain and other CNS tumors by these and other groups, as the etiology and diagnostic patterns of these histopathologies vary significantly. Use of this recode is useful not only for epidemiologic comparisons across populations, but also for investigating potential data collection or diagnostic differences across regions that may affect overall incidence rates of brain and other CNS tumors. While evaluation of brain and other CNS tumors by behavior is common, we encourage the use of the CBTRUS variable across behaviors to fully estimate the burden due to tumors of the brain and other CNS in populations of interest.
Based on the results of the initial evaluation of the CiNA version of the CBTRUS variable, NAACCR has updated the recode to select cases based on the CBTRUS site definition. This variable, CBTRUS Histology Recode, will be included in the June 2023 release of all CiNA data sets and will be available for cancer registries and researchers to calculate on their own data via File*Pro. NAACCR and CBTRUS will collaborate annually on how best to update the variable if coding or groupings change. This variable is intended to be a more dynamic, clinically driven definition for all primary brain and other CNS tumors. Therefore, users of the recode must be mindful that the CBTRUS reporting definition for all primary brain and other CNS tumors is based on a clinical model supported by the various revisions of the WHO Classification of Tumours of the Central Nervous System. Currently, this is reflective of the 2016 WHO CNS classification. As a result, care is needed when using this classification for analyses of data collected prior to 2016, particularly for histopathologic categories (eg, gliomas) that have changed substantially in classification over time.
We encourage cancer registries and researchers to use this variable to ensure more detailed, comprehensive, and clinically relevant assessment of the burden of this important group of tumors.
Footnotes
Funding details for the Central Brain Tumor Registry of the United States can be found at https://www.cbtrus.org.
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