Molecular subgroups of medulloblastoma: the current consensus
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Open Access
- 2 December 2011
- journal article
- research article
- Published by Springer Nature in Acta Neuropathologica
- Vol. 123 (4) , 465-472
- https://doi.org/10.1007/s00401-011-0922-z
Abstract
Medulloblastoma, a small blue cell malignancy of the cerebellum, is a major cause of morbidity and mortality in pediatric oncology. Current mechanisms for clinical prognostication and stratification include clinical factors (age, presence of metastases, and extent of resection) as well as histological subgrouping (classic, desmoplastic, and large cell/anaplastic histology). Transcriptional profiling studies of medulloblastoma cohorts from several research groups around the globe have suggested the existence of multiple distinct molecular subgroups that differ in their demographics, transcriptomes, somatic genetic events, and clinical outcomes. Variations in the number, composition, and nature of the subgroups between studies brought about a consensus conference in Boston in the fall of 2010. Discussants at the conference came to a consensus that the evidence supported the existence of four main subgroups of medulloblastoma (Wnt, Shh, Group 3, and Group 4). Participants outlined the demographic, transcriptional, genetic, and clinical differences between the four subgroups. While it is anticipated that the molecular classification of medulloblastoma will continue to evolve and diversify in the future as larger cohorts are studied at greater depth, herein we outline the current consensus nomenclature, and the differences between the medulloblastoma subgroups.Keywords
This publication has 51 references indexed in Scilit:
- Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomasActa Neuropathologica, 2012
- Development and cancer of the cerebellumTrends in Neurosciences, 2011
- The Genetic Landscape of the Childhood Cancer MedulloblastomaScience, 2011
- Medulloblastoma: clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroupsActa Neuropathologica, 2011
- Subtypes of medulloblastoma have distinct developmental originsNature, 2010
- Identification of a CpG Island Methylator Phenotype that Defines a Distinct Subgroup of GliomaCancer Cell, 2010
- A pathway-based classification of human breast cancerProceedings of the National Academy of Sciences, 2010
- Treatment of Medulloblastoma with Hedgehog Pathway Inhibitor GDC-0449New England Journal of Medicine, 2009
- An investigation of WNT pathway activation and association with survival in central nervous system primitive neuroectodermal tumours (CNS PNET)British Journal of Cancer, 2009
- The 2007 WHO Classification of Tumours of the Central Nervous SystemActa Neuropathologica, 2007