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.