Supratentorial primitive neuroectodermal tumors (stPNETs) and medulloblastomas have long been thought to arise from a common cell type in the subventricular germinal matrix.
Predisposition
PMS2 mutations in syndrome associating cutaneous cafe-au-lait spots (CALS) and early-onset supratentorial primitive neuroectodermal tumor (SPNET) (MIM.608623) (15077197)
CGH
losses
- 1p12-22.1 loss
- 9p loss
gains
- 19p gain
Cytogenetics
9p21 deletion (17592618)
- CDKN2A deletion (17592618)
See also
cPNET (cererebral PNET)
References
Pfister S, Remke M, Toedt G, Werft W, Benner A, Mendrzyk F, Wittmann A, Devens F, von Hoff K, Rutkowski S, Kulozik A, Radlwimmer B, Scheurlen W, Lichter P, Korshunov A. Supratentorial primitive neuroectodermal tumors of the central nervous system frequently harbor deletions of the CDKN2A locus and other genomic aberrations distinct from medulloblastomas. Genes Chromosomes Cancer. 2007 Jun 25; PMID: 17592618