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[Medulloblastoma. Pathology].

Authors
  • Siegfried, A1
  • Delisle, M-B2
  • 1 Département d'anatomie et cytologie pathologiques, institut universitaire du cancer, oncopole, 31059 Toulouse, France; Neuropathologie, laboratoire universitaire d'anatomie et cytologie pathologiques, université Toulouse III-Paul-Sabatier, CHU de Toulouse, 31059 Toulouse, France. , (France)
  • 2 Neuropathologie, laboratoire universitaire d'anatomie et cytologie pathologiques, université Toulouse III-Paul-Sabatier, CHU de Toulouse, 31059 Toulouse, France; Inserm UMR 1214 TONIC, université Toulouse III-Paul-Sabatier, 31059 Toulouse, France. Electronic address: [email protected] , (France)
Type
Published Article
Journal
Neuro-Chirurgie
Publication Date
Apr 24, 2018
Identifiers
DOI: 10.1016/j.neuchi.2017.12.006
PMID: 29703584
Source
Medline
Keywords
Language
French
License
Unknown

Abstract

Medulloblastomas, embryonal neuroepithelial tumors developed in the cerebellum or brain stem, are mainly observed in childhood. The treatment of WHO-Grade IV tumors depends on stratifications that are usually based on postoperative data, histopathological subtype, tumor extension and presence of MYC or NMYC amplifications. Recently, molecular biology studies, based on new technologies (i.e. sequencing, transcriptomic, methylomic) have introduced genetic subtypes integrated into the latest WHO-2016 neuropathological classification. According to this classification, the three genetic groups WNT, SHH, with or without mutated TP53 gene, and non-WNT/non-SHH, comprising subgroups 3 and 4, are recalled in this review. The contribution of immunohistochemistry to define these groups is specified. The four histopathological groups are detailed in comparison to the WHO-2007 classification and the molecular data: classic medulloblastoma, desmoplastic/nodular medulloblastoma, medulloblastoma with extensive nodularity, and large cell/anaplastic medulloblastoma. The groups defined on genetic and histopathological grounds are not strictly concordant. Depending on the age of the patients, their correlations are different, as well as their role in the management and prognosis of these tumors. Other embryonal tumors, for which new classifications are in progress and gliomas may be confused with a medulloblastoma and the elements of the differential diagnosis of these entities are discussed. This evolution in classification fully justifies ongoing structuring procedures such as histopathological review (RENOCLIP) and the organization of molecular biology platforms. Copyright © 2018 Elsevier Masson SAS. All rights reserved.

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