Table 1.
Pathologic subtype | Histologic appearance | Prognosis (% overall survival at 5 years) |
---|---|---|
Classic (71%) | Sheets of uniform small cells with high nuclear to cytoplasmic ratio and round hyperchromatic nuclei. Focal neural differentiation is present in a minority. | WNT subgroup (>95%) SHH subgroup (80%) Group 4 (70%) |
Nodular/desmoplastic (5%) – Conventional (3%) – Paucinodular (1%) – DMEN (<1%) |
Heterogeneous architecture with regions of dense intercellular reticulin and nodules containing tumor cells with a neuronal phenotype and decreased growth fraction. | Infants and children (80%) Adults (80%) p53 mutant tumors (45%) |
Large cell/anaplastic (17%) | Anaplastic tumors have marked pleomorphism, nuclear molding, cell-cell wrapping, and high mitotic activity. Large cell type features spherical cells with round nuclei, open chromatin, and prominent central nucleoli. Large cell and anaplastic types coexist. |
Group 3 (<50%) |
Abbreviations: WNT, wingless; SHH, sonic hedgehog; DMEN, desmoplastic medulloblastoma with excessive nodularity.