Table 3.
Clinical and biologic characteristics of infant and adult SHH medulloblastoma
| Infant SHH medulloblastoma | Adult SHH medulloblastoma | |
|---|---|---|
| Sex | Males = females | Male predominance |
| Age | <4 years | >16 years |
| Tumor location | Vermis | Cerebellar hemispheres |
| Metastasis at diagnosis | Frequently metastatic | Frequently localized |
| Overall survival (5-year) | 85% | 80% |
| Survival by histology | Desmoplastic > classic | No difference |
| Survival by M0 versus M+ | No difference | M0 >M+ |
| Survival by 10q loss (+ or −) | No difference | 10q loss+ < 10q loss− |
| Survival by GLi2 amp (+ or −) | No difference | GLI2 amp+ <GLI2 amp− |
| Gene enrichment | extracellular matrix | HOX, BARHL1, LHX2 |
| Cytogenetics |
CDK 6 amplification (3.6%) 6q loss (0%) 9q loss (56.4%) MycN amplification (12.7%) GLI2 amplification (7.3%) Chr 2 gain (27.3%) Chr 10q loss (54.5%) |
CDK 6 amplification (2.1%) 6q loss (3.1%) 9q loss (37.5%) MycN amplification (1%) GLI2 amplification (6.2%) Chr 2 gain (15.6%) Chr 10q loss (19.8%) |
Note: With kind permission from Springer Science+Business Media: Acta Neuropathol. Pediatric and adult sonic hedgehog medulloblastomas are clinically and molecularly distinct, volume 122, 2011, page 238, Northcott PA, Hielscher T, Dubuc A, et al, Table 3.46 Copyright © 2011.
Abbreviation: SHH, Sonic Hedgehog.