Medulloblastoma.


Journal

Nature reviews. Disease primers
ISSN: 2056-676X
Titre abrégé: Nat Rev Dis Primers
Pays: England
ID NLM: 101672103

Informations de publication

Date de publication:
14 02 2019
Historique:
entrez: 16 2 2019
pubmed: 16 2 2019
medline: 17 9 2019
Statut: epublish

Résumé

Medulloblastoma (MB) comprises a biologically heterogeneous group of embryonal tumours of the cerebellum. Four subgroups of MB have been described (WNT, sonic hedgehog (SHH), Group 3 and Group 4), each of which is associated with different genetic alterations, age at onset and prognosis. These subgroups have broadly been incorporated into the WHO classification of central nervous system tumours but still need to be accounted for to appropriately tailor disease risk to therapy intensity and to target therapy to disease biology. In this Primer, the epidemiology (including MB predisposition), molecular pathogenesis and integrative diagnosis taking histomorphology, molecular genetics and imaging into account are reviewed. In addition, management strategies, which encompass surgical resection of the tumour, cranio-spinal irradiation and chemotherapy, are discussed, together with the possibility of focusing more on disease biology and robust molecularly driven patient stratification in future clinical trials.

Identifiants

pubmed: 30765705
doi: 10.1038/s41572-019-0063-6
pii: 10.1038/s41572-019-0063-6
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

11

Subventions

Organisme : Cancer Research UK
ID : 13457
Pays : United Kingdom

Auteurs

Paul A Northcott (PA)

Department of Developmental Neurobiology, Neurobiology and Brain Tumor Program, St. Jude Children's Research Hospital, Memphis, TN, USA.

Giles W Robinson (GW)

Department of Oncology, Neurobiology and Brain Tumor Program, St. Jude Children's Research Hospital, Memphis, TN, USA.

Christian P Kratz (CP)

Department of Paediatric Haematology and Oncology, Hannover Medical School, Hannover, Germany.

Donald J Mabbott (DJ)

Neurosciences and Mental Health, Hospital for Sick Children, Toronto, Ontario, Canada.

Scott L Pomeroy (SL)

Department of Neurology, Boston Children's Hospital, Boston, MA, USA.
Department of Neurology, Harvard Medical School, Boston, MA, USA.
Eli and Edythe Broad Institute of MIT and Harvard, Cambridge, MA, USA.

Steven C Clifford (SC)

Wolfson Childhood Cancer Research Centre, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK.

Stefan Rutkowski (S)

Department of Paediatric Haematology and Oncology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.

David W Ellison (DW)

Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN, USA.

David Malkin (D)

Division of Haematology/Oncology, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Michael D Taylor (MD)

Developmental and Stem Cell Biology Program, The Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Neurosurgery, Departments of Surgery, Laboratory Medicine and Pathobiology, and of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada.

Amar Gajjar (A)

Department of Oncology, Neurobiology and Brain Tumor Program, St. Jude Children's Research Hospital, Memphis, TN, USA.

Stefan M Pfister (SM)

Hopp Children's Cancer Center Heidelberg (KiTZ), Heidelberg, Germany. s.pfister@kitz-heidelberg.de.
Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Heidelberg, Germany. s.pfister@kitz-heidelberg.de.
Department of Hematology and Oncology, Heidelberg University Hospital, Heidelberg, Germany. s.pfister@kitz-heidelberg.de.

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