PMID- 11807556 OWN - NLM STAT- MEDLINE DCOM- 20020315 LR - 20220318 IS - 0028-0836 (Print) IS - 0028-0836 (Linking) VI - 415 IP - 6870 DP - 2002 Jan 24 TI - Prediction of central nervous system embryonal tumour outcome based on gene expression. PG - 436-42 AB - Embryonal tumours of the central nervous system (CNS) represent a heterogeneous group of tumours about which little is known biologically, and whose diagnosis, on the basis of morphologic appearance alone, is controversial. Medulloblastomas, for example, are the most common malignant brain tumour of childhood, but their pathogenesis is unknown, their relationship to other embryonal CNS tumours is debated, and patients' response to therapy is difficult to predict. We approached these problems by developing a classification system based on DNA microarray gene expression data derived from 99 patient samples. Here we demonstrate that medulloblastomas are molecularly distinct from other brain tumours including primitive neuroectodermal tumours (PNETs), atypical teratoid/rhabdoid tumours (AT/RTs) and malignant gliomas. Previously unrecognized evidence supporting the derivation of medulloblastomas from cerebellar granule cells through activation of the Sonic Hedgehog (SHH) pathway was also revealed. We show further that the clinical outcome of children with medulloblastomas is highly predictable on the basis of the gene expression profiles of their tumours at diagnosis. FAU - Pomeroy, Scott L AU - Pomeroy SL AD - Division of Neuroscience, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA. scott.pomeroy@tch.harvard.edu FAU - Tamayo, Pablo AU - Tamayo P FAU - Gaasenbeek, Michelle AU - Gaasenbeek M FAU - Sturla, Lisa M AU - Sturla LM FAU - Angelo, Michael AU - Angelo M FAU - McLaughlin, Margaret E AU - McLaughlin ME FAU - Kim, John Y H AU - Kim JY FAU - Goumnerova, Liliana C AU - Goumnerova LC FAU - Black, Peter M AU - Black PM FAU - Lau, Ching AU - Lau C FAU - Allen, Jeffrey C AU - Allen JC FAU - Zagzag, David AU - Zagzag D FAU - Olson, James M AU - Olson JM FAU - Curran, Tom AU - Curran T FAU - Wetmore, Cynthia AU - Wetmore C FAU - Biegel, Jaclyn A AU - Biegel JA FAU - Poggio, Tomaso AU - Poggio T FAU - Mukherjee, Shayan AU - Mukherjee S FAU - Rifkin, Ryan AU - Rifkin R FAU - Califano, Andrea AU - Califano A FAU - Stolovitzky, Gustavo AU - Stolovitzky G FAU - Louis, David N AU - Louis DN FAU - Mesirov, Jill P AU - Mesirov JP FAU - Lander, Eric S AU - Lander ES FAU - Golub, Todd R AU - Golub TR LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - England TA - Nature JT - Nature JID - 0410462 SB - IM CIN - Curr Neurol Neurosci Rep. 2003 Mar;3(2):117-9. PMID: 12583839 MH - Adolescent MH - Cerebellar Neoplasms/classification/diagnosis/*genetics/pathology MH - Child MH - Diagnosis, Differential MH - *Gene Expression Profiling MH - Humans MH - Medulloblastoma/classification/diagnosis/*genetics/pathology MH - Oligonucleotide Array Sequence Analysis MH - Outcome Assessment, Health Care MH - Prognosis EDAT- 2002/01/25 10:00 MHDA- 2002/03/16 10:01 CRDT- 2002/01/25 10:00 PHST- 2002/01/25 10:00 [pubmed] PHST- 2002/03/16 10:01 [medline] PHST- 2002/01/25 10:00 [entrez] AID - 415436a [pii] AID - 10.1038/415436a [doi] PST - ppublish SO - Nature. 2002 Jan 24;415(6870):436-42. doi: 10.1038/415436a.