Risk stratification of childhood medulloblastoma in the molecular era: the current consensus

Vijay Ramaswamy, Marc Remke, Eric Bouffet, Simon Bailey, Steven C. Clifford, Francois Doz, Marcel Kool, Christelle Dufour, Gilles Vassal, Till Milde, Olaf Witt, Katja von Hoff, Torsten Pietsch, Paul A. Northcott, Amar Gajjar, Giles W. Robinson, Laetitia Padovani, Nicolas André, Maura Massimino, Barry PizerRoger Packer, Stefan Rutkowski, Stefan M. Pfister, Michael D. Taylor, Scott L. Pomeroy

Research output: Contribution to journalArticle

Abstract

Historical risk stratification criteria for medulloblastoma rely primarily on clinicopathological variables pertaining to age, presence of metastases, extent of resection, histological subtypes and in some instances individual genetic aberrations such as MYC and MYCN amplification. In 2010, an international panel of experts established consensus defining four main subgroups of medulloblastoma (WNT, SHH, Group 3 and Group 4) delineated by transcriptional profiling. This has led to the current generation of biomarker-driven clinical trials assigning WNT tumors to a favorable prognosis group in addition to clinicopathological criteria including MYC and MYCN gene amplifications. However, outcome prediction of non-WNT subgroups is a challenge due to inconsistent survival reports. In 2015, a consensus conference was convened in Heidelberg with the objective to further refine the risk stratification in the context of subgroups and agree on a definition of risk groups of non-infant, childhood medulloblastoma (ages 3–17). Published and unpublished data over the past 5 years were reviewed, and a consensus was reached regarding the level of evidence for currently available biomarkers. The following risk groups were defined based on current survival rates: low risk (>90 % survival), average (standard) risk (75–90 % survival), high risk (50–75 % survival) and very high risk (

Original languageEnglish
Pages (from-to)1-11
Number of pages11
JournalActa Neuropathologica
DOIs
Publication statusAccepted/In press - Apr 4 2016

Keywords

  • Genomics
  • Group 3
  • Group 4
  • Medulloblastoma
  • Outcomes
  • p53
  • SHH
  • Subgroups
  • WNT

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Cellular and Molecular Neuroscience

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    Ramaswamy, V., Remke, M., Bouffet, E., Bailey, S., Clifford, S. C., Doz, F., Kool, M., Dufour, C., Vassal, G., Milde, T., Witt, O., von Hoff, K., Pietsch, T., Northcott, P. A., Gajjar, A., Robinson, G. W., Padovani, L., André, N., Massimino, M., ... Pomeroy, S. L. (Accepted/In press). Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. Acta Neuropathologica, 1-11. https://doi.org/10.1007/s00401-016-1569-6