Medulloblastomas in adults: prognostic factors and lessons from paediatrics.

Détails

ID Serval
serval:BIB_C058570CD481
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Medulloblastomas in adults: prognostic factors and lessons from paediatrics.
Périodique
Current Opinion in Neurology
Auteur(s)
Nay Fellay C., Frappaz D., Sunyach M.P., Franceschi E., Brandes A.A., Stupp R.
ISSN
1473-6551 (Electronic)
ISSN-L
1080-8248
Statut éditorial
Publié
Date de publication
2011
Volume
24
Numéro
6
Pages
626-632
Langue
anglais
Notes
Publication types: Journal ArticlePublication Status: ppublish
Résumé
Purpose of reviewMedulloblastomas are very rare in adults. Usual treatment consists of craniospinal radiation with or without chemotherapy. Current efforts focus on a better understanding of tumour biology, stratifying patients into risk groups and adapting treatment accordingly. This review discusses clinical and new molecular risk factors that will help to optimize treatment in adult medulloblastoma patients.Recent findingsThe clinical risk stratification should be complemented with new molecular prognostic markers. Gene-expression profiling has permitted identification of four to six molecular medulloblastoma subgroups. The WNT subgroup shows overexpression of genes of the WNT/wingless signalling pathway with frequent mutations of the CNNTB1 gene, loss of chromosome 6 and accumulation of nuclear beta-catenin, and is most often seen in children with medulloblastomas of classical histology. This variant has a good prognosis. Activation of the sonic hedgehog pathway with frequent mutations of the PTCH and SUFU genes, loss of 9q, and positivity for GLI1 and SFRP1 is more frequent in children less than 3 years old and in adults, commonly associated with desmoplastic histology. Other subgroups are not so well defined and have overlapping characteristics, but MYC/MYCN amplification, 17q gain and, large cell/anaplastic histology are factors of poor prognosis.SummaryNew molecular subgroups will help tailor treatment and further develop new targeted therapies. Prospective and ideally randomized trials should be performed in adults, including risk stratification by molecular markers, to identify optimal treatment for each risk group.
Pubmed
Web of science
Création de la notice
08/12/2011 13:02
Dernière modification de la notice
03/03/2018 21:05
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