Table 1.
WNT | SHH | Group 3 | Group 4 | |
---|---|---|---|---|
Prevalence | 10% | 30% | 25% | 35% |
Age | Children, teens | Infants, adults | Infants, children | Infants, children, adults |
Sex (M:F) | 1:1 | 1:1 | 2:1 | 3:1 |
Histology | Classic | Nodular desmoplastic histology, classic, LCA | Classic, LCA | Classic, LCA |
Metastasis | Low | Low | High | High |
Recurrence | Rare | Local | Metastasis | Metastasis |
Prognosis | Best | Intermediate | Poor | Intermediate |
5-Year OS | 95% | 75% | 50% | 75% |
Genetics | CTNNB1 DDX3X SMARCA4 | MYCN, GLI2, PTCH1, SUFU, MLL2, SMO, TP53, BCOR1, LDB1, GABRG1 | MYC, PVT1, OTX2, MLL2, SMARCA4, CHD7 | OTX2, DDX31, CHD7, SNCAIP, MYCN, CDK6 GFI1/GFI1B, MLL2, KDM6A, MLL3, ZMYM3 |
Chromosome | chr 6 loss | chr 3q gain, chr 9q loss, 10q loss | chr 1q gain, chr 5q loss, 10q loss | Isochromosome 17q chr X loss, 17p loss |
Cells of origin | Lower rhombic lip progenitors | Cerebellar granule neuron precursors | Neural stem cells? | Upper rhombic lip progenitors |
LCA: large cell anaplastic.