This is a corrigendum to: J Bryan Iorgulescu, Chuxuan Sun, Corey Neff, Gino Cioffi, Catherine Gutierrez, Carol Kruchko, Jennifer Ruhl, Kristin A Waite, Serban Negoita, Jim Hofferkamp, Tarik Tihan, Roger McLendon, Daniel J Brat, Quinn T Ostrom, Jill S Barnholtz-Sloan, Molecular biomarker-defined brain tumors: Epidemiology, validity, and completeness in the United States, Neuro-Oncology, Volume 24, Issue 11, November 2022, Pages 1989–2000, https://doi.org/10.1093/neuonc/noac113
In the originally published version of this manuscript, in Table 3 of the above manuscript, an error was identified with how the table was generated from the underlying databases that resulted in small errors (each <1% in magnitude) in the frequencies and corresponding percentages of: total cases, age-adjusted incidence, sex, and race/ethnicity for several tumor types. Additionally affected were the median ages at diagnoses (with interquartile ranges [IQR]) for these tumor types. Notably, the correct median age at diagnosis for WHO grade 4 IDH-mutant astrocytomas is: 47 years (IQR: 36-60). This error also affected the total count of “Glioblastoma” in Supplemental Table 1, resulting in an error of <3%. The remaining analyses of the manuscript were unaffected, the manuscript’s text was unaffected, and the manuscript’s conclusions remain unchanged. The corrected Tables are displayed below, with the corrected results denoted with bolding. The authors apologize for this error that has now been corrected.
Table 3.
Annual age-adjusted incidence, median age, sex, and race/ethnicity of molecularly-defined brain tumors from U.S. central cancer registries for diagnosis year 2018
| Tumor type | ICD-O-3 histology codes | Gradea | Total cases | Age-adjusted incidence per 100,000 (95% CI) | Age (median, interquartile range) | Sexg | Race/Ethnicityf | ||
|---|---|---|---|---|---|---|---|---|---|
| Female (%) | White non-Hispanic (%) | Black non-Hispanic (%) | Hispanic (%) | ||||||
| Adult-type diffuse glioma | |||||||||
| IDHmut Astrocytoma (BMM 1, 3) |
9400/3 9401/3 9445/3 | 2 | 425 | 0.14 (0.12-0.15) | 34 (27, 45) | 41.6% | 80.5% | 5.0% | 10.2% |
| 3 | 465 | 0.15 (0.14-0.16) | 37 (29, 48) | 44.7% | 82.2% | 5.4% | 9.5% | ||
| 4 | 241 | 0.07 (0.06-0.08) | 47 (36, 60) | 42.3% | 80.1% | 8.5% | 9.3% | ||
| IDHwt Astrocytoma and Glioblastomab (BMM 2, 4, 5) |
9400/3 9401/3 9440/3 | 2 | 190 | 0.05 (0.05-0.06) | 54 (32, 66) | 47.9% | 78.2% | -- | -- |
| 3 | 369 | 0.10 (0.09-0.11) | 59 (47, 70) | 45.5% | 84.4% | 6.6% | 5.7% | ||
| 4 | 6,878 | 1.73 (1.69-1.78) | 65 (56, 72) | 40.2% | 83.2% | 6.0% | 8.3% | ||
| IDHmut & 1p/19q- codeleted Oligodendroglioma (BMM 6,7) |
9450/3 9451/3 |
2 | 437 | 0.14 (0.13-0.15) | 42 (33, 54) | 47.6% | 76.0% | 5.6% | 14.0% |
| 3 | 274 | 0.08 (0.07-0.09) | 48 (37, 57) | 46.0% | 76.4% | -- | -- | ||
| Medulloblastoma | |||||||||
| SHH-activated & TP53wt (BMM 8) | 9471/3 | 76 | 0.03 (0.02-0.03) | 21 (6, 29) | 36.8% | 60.3% | -- | -- | |
| SHH-activated & TP53mut | 9476/3 | <16 | n/ac | -- | -- | -- | -- | -- | |
| WNT-activated | 9475/3 | <16 | n/ac | -- | -- | -- | -- | -- | |
| nonWNT/nonSHH | 9477/3 | 47 | n/ac | 7 (4, 10) | 42.6% | 52.3% | -- | 36.4% | |
| Other tumor types d , e | |||||||||
| Diffuse midline glioma, H3 K27M-mutant | 9385/3 | 144 | 0.05 (0.04-0.06) | 14 (7, 28) | 53.5% | 58.2% | -- | 22.7% | |
| ETMR C19MC-altered (BMM 9) | 9478/3 | <16 | <0.01 | -- | -- | -- | -- | -- | |
| RELA-fusion ependymoma | 9396/3 | <16 | <0.01 | -- | -- | -- | -- | -- |
Data provided by CDC’s National Program of Cancer Registries and NCI’s Surveillance, Epidemiology and End Results Program, November 2020 submissions.
-- Suppressed if case counts are <16
BMM = brain molecular markers variable, CI = confidence interval, mut = mutant, wt = wildtype, ETMR = embryonal tumor with multilayered rosettes
aAdult-type diffuse glioma cases reported as WHO grade 1 or “low-grade, NOS” were grouped with WHO grade 2.
bIn WHO-CNS5, all IDH-wildtype adult-type diffuse astrocytic gliomas are classified as glioblastoma, IDH-wildtype, WHO CNS grade 4, without separate grades 2 or 3.
cBoth histologically-defined and new molecularly-defined ICD-O-3 codes for medulloblastomas were reported in the registry data; however, only a single ICD-O-3 diagnosis can be reported per case. As a result, the national incidence rates could only be estimated for BMM-coded SHH-activated & TP53-wildtype subtype.
dThe implementation of updated ICD-O-3 codes in 2018 also included 9509/1, which groups together three distinct glioneuronal and neuronal tumor types: papillary glioneuronal tumor, rosette-forming glioneuronal tumor, diffuse leptomeningeal glioneuronal tumor. Altogether, these three diagnoses were reported in 51 patients and associated with an AAIR of 0.02 (95%CI: 0.01-0.02), median age at diagnosis of 24 years-old (interquartile range 13-39), with 52.9% in females.
eIn 2018 a separate ICD-O-3 code was introduced for pilomyxoid astrocytomas (9425/3)--a subtype of pilocytic astrocytomas that is thought to be more aggressive in behavior. Cases presented at a median age of 3 years-old (interquartile range 1-7; n=29).
fFor race/ethnicity, patients with Asian/Pacific Islander, other, or unknown races/ethnicities were suppressed due to low cell counts.
g% males = 100% - % females.
Supplementary Table 1.
Availability and frequency of reported pathological grade by ICD-O-3 coded tumor type, from U.S. central cancer registries for diagnosis year 2018
| Tumor type | ICD-O-3 | Total | Unvailablea n (%) |
Available n (%) |
Grade for cases with available grade information | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| WHO CNS grade | Low-grade (L) n (%) |
High-grade (H) n (%) |
||||||||
| 1 | 2 | 3 | 4 | |||||||
| n (%) | n (%) | n (%) | n (%) | |||||||
| Adult-type diffuse gliomas | ||||||||||
| ‘Oligodendroglioma, NOS’ | 9450/3 | 655 | 136 (21%) | 519 (79%) | -- | 88% | 9% | -- | -- | -- |
| ‘Anaplastic’ oligodendroglioma | 9451/3 | 321 | 54 (17%) | 267 (83%) | 0% | -- | 93% | -- | -- | -- |
| ‘Diffuse’ astrocytoma | 9400/3 | 1,304 | 442 (34%) | 862 (66%) | 2% | 73% | 10% | 7% | 5% | 3% |
| ‘Anaplastic’ astrocytoma | 9401/3 | 1,363 | 418 (31%) | 945 (69%) | -- | 3% | 88% | 8% | 0% | -- |
| *Glioblastoma, IDH-mutant | 9445/3 | 237 | 30 (13%) | 207 (87%) | 0% | -- | -- | 97% | 0% | -- |
| Glioblastoma | 9440/3 | 11,332 | 2,865 (25%) | 8,467 (75%) | -- | -- | 0.4% | 98% | -- | 1.9% |
| Giant cell glioblastoma | 9441/3 | 83 | -- | -- | -- | -- | -- | 99% | -- | -- |
| Gliosarcoma | 9442/3 | 238 | 28 (12%) | 210 (88%) | 0% | -- | 0% | 95% | -- | -- |
| Malignant glioma, NOS | 9380/3 | 625 | 308 (49%) | 317 (51%) | 6% | 14% | 13% | 21% | 22% | 24% |
| Pediatric-type diffuse low-grade gliomas | ||||||||||
| Angiocentric glioma | 9431/1 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Pediatric-type diffuse high-grade gliomas | ||||||||||
| *Diffuse midline glioma, H3 K27M-mutant | 9385/3 | 144 | 72 (50%) | 72 (50%) | -- | -- | -- | 94% | -- | -- |
| Circumscribed astrocytic gliomas | ||||||||||
| Pilocytic astrocytoma | 9421/1, 9421/3 | 918 | 161 (18%) | 757 (82%) | 95% | 2% | -- | -- | 3% | -- |
| *Pilomyxoid astrocytoma | 9425/3 | 28 | -- | -- | -- | -- | 0% | 0% | -- | 0% |
| Pleomorphic xanthoastrocytoma | 9424/3 | 100 | -- | -- | -- | 60% | 36% | 0% | -- | 0% |
| Subependymal giant cell astrocytoma | 9384/1 | 23 | -- | -- | -- | 0% | 0% | 0% | 0% | 0% |
| Choroid glioma | 9444/1 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Astroblastoma | 9430/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Glioneuronal and neuronal tumors | ||||||||||
| Ganglioglioma | 9505/1 | 373 | 88 (24% | 285 (76%) | 90% | -- | 0% | -- | 8.4% | 0% |
| Anaplastic ganglioglioma | 9505/3 | 28 | -- | -- | -- | -- | -- | -- | 0% | 0% |
| Desmoplastic infantile astrocytoma and ganglioglioma | 9412/1 | 25 | -- | -- | -- | -- | -- | -- | -- | -- |
| Dysembryoplasticneuroepithelialtumor | 9413/0 | 135 | 32 (24%) | 103 (76%) | 86% | 0% | 0% | 0% | -- | -- |
| Papillary glioneuronal tumor OR Rosette-forming glioneuronal tumor OR Diffuse leptomeningeal glioneuronal tumor | 9509/1 | 51 | -- | -- |
84% | 0% | -- | 0% | -- | -- |
| Gangliocytoma | 9492/0 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) | 9493/0 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Central neurocytoma OR extraventricular neurocytoma OR Cerebellar liponeurocytoma | 9506/1 | 82 | 22 (27%) | 60 (73%) | -- | 98% | -- | -- | -- | -- |
| Ependymal tumors | ||||||||||
| Ependymoma, NOS | 9391/3 | 503 | 99 (20%) | 404 (80%) | -- | 91% | -- | 0% | -- | -- |
| Anaplastic ependymoma | 9392/3 | 145 | 23 (16%) | 122 (84%) | 0% | -- | 92% | -- | 0% | -- |
| *Ependymoma, RELA (ZFTA) fusion-positive | 9396/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Myxopapillary ependymoma | 9394/1 | 267 | 55 (21%) | 212 (79%) | 95% | -- | 0% | -- | 0% | 0% |
| Subependymoma | 9383/1 | 174 | 32 (18%) | 142 (82%) | 89% | -- | -- | 0% | -- | 0% |
| Choroid plexus tumors | ||||||||||
| Choroid plexus papilloma | 9390/0 | 102 | 30 (29%) | 72 (71%) | 94% | -- | 0% | 0% | -- | 0% |
| Atypical choroid plexus papilloma | 9390/1 | 23 | -- | -- | -- | 95% | -- | -- | -- | -- |
| Choroid plexus carcinoma | 9390/3 | 22 | -- | -- | -- | 0% | -- | 0% | 0% | -- |
| Embryonal tumors | ||||||||||
| Medulloblastoma, NOS | 9470/3 | 211 | 38 (18%) | 173 (82%) | -- | 0% | -- | 98% | 0% | -- |
| Desmoplastic/nodular medulloblastoma | 9471/3 | 104 | -- | -- | 0% | 0% | 0% | 100% | 0% | 0% |
| Large cell medulloblastoma | 9474/3 | 36 | -- | -- | 0% | 0% | 0% | 100% | 0% | 0% |
| *Medulloblastoma, WNT-activated | 9475/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| *Medulloblastoma, SHH-activated & TP53-mutant | 9476/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| *Medulloblastoma, non-WNT/non-SHH | 9477/3 | 51 | -- | -- | 0% | 0% | 0% | 100% | 0% | 0% |
| Atypical teratoid/rhabdoid tumor | 9508/3 | 77 | 17 (22%) | 60 (78%) | 0% | -- | -- | 98% | 0% | 0% |
| Other CNS embryonal tumor (CNS Neuroblastoma, Medulloepithelioma, Embryonal tumor with multilayered rosettes) |
9500/3, 9501/3, 9473/3, 9478/3 |
69 | 38 (55%) | 31 (45%) | 0% | 0% | -- | 90% | 0% | -- |
| Pineal tumors | ||||||||||
| Pineocytoma | 9361/1 | 29 | -- | -- | -- | -- | -- | 0% | 0% | 0% |
| Pineal parenchymal tumor of intermediate differentiation | 9361/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Pineoblastoma | 9362/3 | 68 | 28 (41%) | 40 (59%) | 0% | -- | -- | 60% | 0% | -- |
| Papillary tumor of pineal region | 9395/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Cranial and spinal nerve tumors | ||||||||||
| Schwannoma | 9560/0 | 3,163 | 1,786 (56%) | 1,377 (44%) | 99% | -- | 0% | -- | -- | 0% |
| Neurofibroma | 9540/0 | 57 | 33 (58%) | 24 (42%) | 100% | 0% | 0% | 0% | 0% | 0% |
| Perineurioma | 9571/0,3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Malignant peripheral nerve sheath tumor OR melanotic MPNST | 9540/3 | 17 | -- | -- | 0% | 0% | -- | 0% | 0% | -- |
| Paraganglioma | 8680/0-3 8694/1, 8693/3 | 44 | 24 (55%) | 20 (45%) | 95% | 0% | 0% | 0% | -- | -- |
| Meningiomas | ||||||||||
| Meningioma (including meningothelial, fibrous, psammomatous, angiomatous, hemangioblastic, transitional, secretory, microcystic, metaplastic, lymphoplasmacytic-rich) | 9530/0, 9531/0, 9532/0, 9533/0, 9534/0, 9535/0, 9537/0 | 10,408 | 2,843 (27%) | 7,565 (72%) | 94% | 5.4% | -- | -- | -- | 0% |
| Atypical meningioma | 9539/1 | 1,546 | 195 (13%) | 1,351 (87%) | 4.5% | 95% | -- | -- | -- | 0% |
| Clear cell OR Chordoid meningioma | 9538/1 | 115 | 24 (21%) | 91 (79%) | -- | 99% | -- | 0% | 0% | 0% |
| Anaplastic (malignant) meningioma | 9530/3 | 204 | 35 (17%) | 169 (83%) | 12% | 10% | 76% | -- | -- | 0% |
| Papillary OR Rhabdoid meningioma | 9538/3 | <16 | -- | -- | -- | -- | -- | -- | -- | -- |
| Mesenchymal, non-meningothelialtumors | ||||||||||
| Benign SFT | 9150/1 | 39 | -- | -- | -- | -- | -- | 0% | 0% | 0% |
| Malignant SFT | 9150/3 | 45 | -- | -- | 0% | -- | 81% | 0% | 0% | 0% |
| *SFT grade1 | 8815/0 | 30 | -- | -- | -- | -- | -- | 0% | 0% | 0% |
| *SFT grade 2 | 8815/1 | 20 | -- | -- | 0% | 100% | 0% | 0% | 0% | 0% |
| *SFT grade 3 | 8815/3 | 25 | -- | -- | -- | -- | -- | -- | -- | -- |
| Hemangioblastoma | 9161/1 | 490 | 156 (32%) | 334 (68%) | 78% | 9.6% | 12% | -- | -- | 0% |
| Tumors of the sellar region | ||||||||||
| Craniopharyngioma (including adamantinomatous, papillary) | 9350/1, 9351/1, 9352/1 | 507 | 227 (45%) | 280 (55%) | 99% | -- | 0% | 0% | -- | 0% |
| *Pituicytoma, Granular cell tumor of the sellar region, spindle cell oncocytoma | 9582/0, 9432/1, 8290/0 | 16 | -- | -- | -- | 0% | 0% | 0% | 0% | 0% |
Data provided by CDC’s National Program of Cancer Registries and NCI’s Surveillance, Epidemiology and End Results Program, November 2020 submissions.
tNew ICD-O-3 term and code implemented for cases diagnosed on January 1, 2018 and after.
aPathological grade was reported for cases the underwent definitive resection. Unavailable pathological grade was defined as codes A-D or 9.
-- Suppressed if case counts are <16 or if suppressed cells are able to be otherwise derived.
Contributor Information
J Bryan Iorgulescu, Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School; Department of Medical Oncology, Dana-Farber Cancer Institute.
Chuxuan Sun, Department of Biostatistics, Duke University School of Medicine.
Corey Neff, Central Brain Tumor Registry of the United States; Department of Neurosurgery, Duke University School of Medicine.
Gino Cioffi, Central Brain Tumor Registry of the United States; Trans Divisional Research Program, Division of Cancer Epidemiology and Genetics, National Cancer Institute.
Catherine Gutierrez, Department of Pathology, Brigham and Women’s Hospital, Harvard Medical School; Department of Medical Oncology, Dana-Farber Cancer Institute.
Carol Kruchko, Central Brain Tumor Registry of the United States.
Jennifer Ruhl, Surveillance, Epidemiology, and End Results program, National Cancer Institute.
Kristin Waite, Central Brain Tumor Registry of the United States; Trans Divisional Research Program, Division of Cancer Epidemiology and Genetics, National Cancer Institute.
Serban Negoita, Surveillance, Epidemiology, and End Results program, National Cancer Institute.
Jim Hofferkamp, North American Association of Central Cancer Registries.
Tarik Tihan, Division of Neuropathology, Department of Pathology, University of California San Francisco.
Roger McLendon, Department of Pathology, Duke University School of Medicine; The Preston Robert Tisch Brain Tumor Center, Duke University School of Medicine; Duke Cancer Institute, Duke University Medical Center.
Daniel J Brat, Department of Pathology, Northwestern University Feinberg School of Medicine.
Quinn T Ostrom, Central Brain Tumor Registry of the United States; Department of Neurosurgery, Duke University School of Medicine; The Preston Robert Tisch Brain Tumor Center, Duke University School of Medicine; Duke Cancer Institute, Duke University Medical Center.
Jill S Barnholtz-Sloan, Central Brain Tumor Registry of the United States; Trans Divisional Research Program, Division of Cancer Epidemiology and Genetics, National Cancer Institute; Center for Biomedical Informatics & Information Technology, National Cancer Institute.