Molecular subgroups of medulloblastoma: the current consensus

By Michael D. Taylor, P. Northcott, A. Korshunov, M. Remke, Yoon-Jae Cho, S. Clifford, C. Eberhart, D. W. Parsons, S. Rutkowski, A. Gajjar and 6 more. Acta Neuropathologica, 2011

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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.

Subfields: Cancer Research, Genetics, Molecular Biology

View Paper: https://www.semanticscholar.org/paper/82d950bbd43e6611fd5d61d766a606f63008e682

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Citations in the corpus

Molecular subgroups of medulloblastoma: an international meta-analysis of transcriptome, genetic aberrations, and clinical data of WNT, SHH, Group 3, and Group 4 medulloblastomas (2012, background): “However, the current consensus in the medulloblastoma ﬁeld is that there are only four core molecular subgroups in medulloblastoma, as recently agreed upon at a consensus meeting in Boston (see also the manuscript by Taylor et al. [24] in this issue).”
Genome Sequencing of SHH Medulloblastoma Predicts Genotype-Related Response to Smoothened Inhibition (2014, background): “They are highly distinct in tumor cell histology and biology, and in addition show divergent clinical phenotypes such as patient demographics, tumor dissemination, and patient outcome (Kool et al., 2012; Northcott et al., 2012a; Taylor et al., 2012).”
The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary (2016, background): “, desmoplastic/nodular, medulloblastoma with extensive nodularity, large cell, and anaplastic) and it is now widely accepted that there are four genetic (molecular) groups of medulloblastoma: WNTactivated, SHH-activated, and the numerically designated “group 3” and “group 4” [46].”
Molecular Classification of Ependymal Tumors across All CNS Compartments, Histopathological Grades, and Age Groups. (2015, background): “A robust and uniform (epi)genetic classification of ependymal tumors as presented herein may guide researchers, neuropathologists, and clinicians to a better understanding of the heterogeneity of this disease, analogous to (epi)genetic subgroups of medulloblastoma (Kool et al., 2012; Northcott et al”
The whole-genome landscape of medulloblastoma subtypes (2017, neutral): “epigenetic signatures that define clinically relevant patient subsets 12,13 .”