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supratentorial primitive neuroectodermal tumor

Wednesday 27 June 2007

Supratentorial primitive neuroectodermal tumors (stPNETs) and medulloblastomas have long been thought to arise from a common cell type in the subventricular germinal matrix.


- PMS2 mutations in syndrome associating cutaneous cafe-au-lait spots (CALS) and early-onset supratentorial primitive neuroectodermal tumor (SPNET) (MIM.608623) (15077197)


- losses

  • 1p12-22.1 loss
  • 9p loss

- gains

  • 19p gain


- 9p21 deletion (17592618)

See also

- cPNET (cererebral PNET)


- 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