Table 7.
Total Cases, Average Annual Age-Specific Incidencea, Median Age, Sex, and Race/Ethnicity of Molecularly-Defined Brain and Other Central Nervous System Tumors in Children and Adolescents Ages 0–19 Years, CBTRUS Childhood and Adolescent Report: NPCR and SEER, 2018–2019
| Tumor type | ICD-O-3 histopathology codes | Total New Cases (2018–2019) | Rate (95% CI) | Age (median, interquartile range) | Female (%) | Non-Hispanic White (%) | Non-Hispanic Black (%) | Hispanic (%) |
|---|---|---|---|---|---|---|---|---|
| Adult-type diffuse glioma | ||||||||
| IDH1/2 - mutant Astrocytomab | 9400/3, 9401/3, 9445/3 | 142 | 0.09 (0.07–0.10) | 15 (8.75–18) | 45.8 | 70.4 | -- | 16.2 |
| IDH1/2 - wildtype Astrocytoma and Glioblastomac | 9400/3, 9401/3, 9440/3 | 279 | 0.17 (0.15–0.19) | 10 (6–15) | 43.0 | 67.7 | 10.0 | 16.5 |
| IDH1/2 mutant & 1p/19q-codeleted Oligodendrogliomad | 9450/3, 9451/3 | 34 | 0.02 (0.01–0.03) | 15 (9.5–18) | -- | 82.4 | -- | -- |
| Medulloblastoma | ||||||||
| SHH-activated & TP53 wildtypee | 9471/3 | 80 | 0.05 (0.04–0.06) | 4.5 (2–9.25) | 31.3 | 51.2 | -- | 21.2 |
| SHH-activated & TP53 mutant | 9476/3 | < 16 cases | -- | -- | -- | -- | -- | -- |
| WNT-activated | 9475/3 | 25 | 0.02 (0.01–0.02) | 9 (7–11) | -- | 72.0 | -- | -- |
| nonWNT/nonSHH | 9477/3 | 122 | 0.08 (0.06–0.09) | 7 (4–10) | 34.4 | 63.1 | -- | 26.2 |
| Other tumor types | ||||||||
| Embryonal tumor with multilayered rosettes, C19MC-alteredf | 9478/3 | 25 | 0.02 (0.01–0.02) | 2 (1–3) | -- | -- | -- | |
| Ependymoma, RELA fusion | 9396/3 | < 16 cases | -- | -- | -- | -- | -- | |
| Diffuse midline glioma, H3 K27M-mutant | 9385/3 | 197 | 0.12 (0.11–0.14) | 8 (5–12) | 59.9 | 49.2 | 11.7 | 27.9 |
| Pilomyxoid astrocytoma | 9425/3 | 40 | 0.02 (0.02–0.03) | 3 (1–7) | 45.0 | 72.5 | -- | -- |
aRates are per 100,000.
bICD-O-3 histopathology code of 9400/3, 9401/3 and a BMM value of 1 or 3, or ICD-O-3 histopathology code of 9445/3.
cICD-O-3 histopathology code of 9400/3, 9401/3, 9440/3 and a BMM value of 2, 4, or 5.
dICD-O-3 histopathology code of 9450/3, 9451/3 and a BMM of 6 or7.
eICD-O-3 histopathology code of 9471/3 and a BMM value of 8.
fICD-O-3 histopathology code of 9478/3 and a BMM value of 9.
Data are not presented when fewer than 16 cases were reported for the specific category. Counts and associated rates cannot be provided when Total Cases (2018–2019) are fewer than 16 cases or when a value based on less than 16 cases can be back-calculated using a cell. Suppressed cases are included in the total count.
Abbreviations: BMM, brain molecular markers variable; 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.