Table 4.
Average Annual Age-Adjusted Incidence Ratesa, Median Age at Diagnosis, Sex, and Race/Ethnicity of Histopathologically-Confirmed Molecularly-Defined Brain and Other Central Nervous System Tumors by WHO Gradeb for Diagnosis Years 2018-2020, CBTRUS Statistical Report: US Cancer Statistics – NPCR and SEER, 2018-2020
| Histopathology | ICD-O-3 Histopathology Codes | WHO Grade | Total cases (2018-2020)c | Rate (95% CI) | Age (median, interquartile range) | Female (%) | Non-Hispanic White (%) | Non-Hispanic Black (%) | Hispanic (%) |
|---|---|---|---|---|---|---|---|---|---|
| Adult-type diffuse glioma | |||||||||
| IDH-mutant Astrocytoma (BMM 1, 3) |
9400/3, 9401/3, 9445/3 | All grades | 4,293 | 0.45 (0.43-0.46) | 37 (29-49) | 42.5% | 78.7% | 6.5% | 11.8% |
| 2/IId | 1,238 | 0.13 (0.13-0.14) | 35 (28-45) | 40.8% | 78.5% | 6.4% | 11.2% | ||
| 3/III | 1,403 | 0.15 (0.14-0.16) | 37 (29-49) | 43.0% | 79.6% | 5.9% | 11.8% | ||
| 4/IV | 933 | 0.09 (0.09-0.10) | 39 (31-54) | 42.0% | 78.2% | 7.1% | 12.0% | ||
| IDH-wildtype Astrocytoma and Glioblastomade (BMM 2, 4, 5) |
9400/3, 9401/3, 9440/3 | All grades | 31,971 | 2.66 (2.63-2.69) | 65 (56-72) | 41.5% | 82.3% | 6.4% | 8.7% |
| 2/II | 578 | 0.05 (0.05-0.06) | 52 (33-65) | 44.8% | 77.6% | 9.2% | 9.7% | ||
| 3/III | 1,090 | 0.10 (0.09-0.10) | 60 (46-70) | 46.2% | 80.9% | 7.3% | 8.7% | ||
| 4/IV | 23,088 | 1.91 (1.89-1.94) | 65 (56-72) | 40.6% | 82.4% | 6.4% | 8.7% | ||
| IDH-mutant & 1p/19q-codeleted Oligodendroglioma (BMM 6, 7) |
All grades | 2,783 | 0.29 (0.28-0.30) | 45 (35-56) | 43.6% | 77.4% | 4.7% | 13.9% | |
| 9450/3, 9451/3 | 2/II | 1,411 | 0.15 (0.14-0.16) | 42 (34-53) | 43.4% | 75.5% | 5.0% | 15.0% | |
| 3/III | 928 | 0.09 (0.09-0.10) | 48 (37-58) | 42.7% | 79.2% | 4.6% | 12.6% | ||
| Medulloblastoma f | |||||||||
| SHH-activated & TP53-wildtype (BMM 8) | 9471/3 | All grades | 244 | 0.03 (0.02-0.03) | 19 (5-31) | 37.7% | 57.0% | 10.6% | 28.1% |
| SHH-activated & TP53-mutant | 9476/3 | All grades | 28 | 0.00 (0.00-0.00) | 11 (7.5-23.25) | -- | -- | -- | -- |
| WNT-activated | 9475/3 | All grades | 47 | 0.01 (0.00-0.01) | 10 (7-12.5) | -- | 61.7% | -- | -- |
| Non-WNT/non-SHH | 9477/3 | All grades | 208 | 0.02 (0.02-0.03) | 8 (4-12) | 33.7% | 61.5% | -- | 30.0% |
| Other tumor types | |||||||||
| Diffuse midline glioma, H3 K27M-mutant | 9385/3 | All grades | 527 | 0.06 (0.05-0.06) | 15 (7-33) | 52.2% | 57.6% | 13.0% | 23.4% |
| ETMR C19MC-altered (BMM 9) | 9478/3 | All grades | 45 | 0.01 (0.00-0.01) | 2 (2-3) | 57.8% | 64.4% | -- | -- |
| RELA-fusion ependymoma | 9396/3 | All grades | 23 | 0.00 (0.00-0.00) | 13 (4.5-23.5) | -- | -- | -- | -- |
aRates are per 100,000 and are age-adjusted to the 2000 US standard population.
bWHO grade is reported according to 2016 WHO classification, in which Roman numerals are used to denote tumor grade.
cExcludes cases with missing molecular classification data or that are not histopathologically-confirmed.
dAdult-type diffuse glioma cases reported as WHO grade I or “low-grade, NOS” were grouped with WHO grade II.
eIn WHO-CNS5, grading is denoted using Arabic numerals rather than roman numerals. In this 2021 revision, all IDH-wildtype adult-type diffuse astrocytic gliomas are classified as glioblastoma, IDH-wildtype, WHO CNS grade 4, without separate grades 2 or 3.
f Both histopathologically-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 not be estimated for the SHH-activated and TP53 mutant subtype.
-- 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; NOS, not otherwise specified; ETMR, Embryonal tumor with multilayered rosettes.