TY - JOUR
T1 - Molecular subgroups of medulloblastoma
T2 - The current consensus
AU - Taylor, Michael D.
AU - Northcott, Paul A.
AU - Korshunov, Andrey
AU - Remke, Marc
AU - Cho, Yoon Jae
AU - Clifford, Steven C.
AU - Eberhart, Charles G.
AU - Parsons, D. Williams
AU - Rutkowski, Stefan
AU - Gajjar, Amar
AU - Ellison, David W.
AU - Lichter, Peter
AU - Gilbertson, Richard J.
AU - Pomeroy, Scott L.
AU - Kool, Marcel
AU - Pfister, Stefan M.
PY - 2012/4
Y1 - 2012/4
N2 - 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.
AB - 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.
KW - Consensus
KW - Group 3
KW - Group 4
KW - Medulloblastoma
KW - SHH
KW - Subgroups
KW - WNT
UR - http://www.scopus.com/inward/record.url?scp=84860821444&partnerID=8YFLogxK
U2 - 10.1007/s00401-011-0922-z
DO - 10.1007/s00401-011-0922-z
M3 - Article
C2 - 22134537
AN - SCOPUS:84860821444
SN - 0001-6322
VL - 123
SP - 465
EP - 472
JO - Acta Neuropathologica
JF - Acta Neuropathologica
IS - 4
ER -