Medulloblastoma

doi:10.1038/s41572-019-0063-6

Medulloblastoma

2019-02-14

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DOI：10.1038/s41572-019-0063-6

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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.

Keywords:

CENTRAL-NERVOUS-SYSTEM

STANDARD-RISK MEDULLOBLASTOMA

DOSE CRANIOSPINAL IRRADIATION

PROSPECTIVE RANDOMIZED-TRIAL

EARLY-CHILDHOOD MEDULLOBLASTOMA

POSTERIOR-FOSSA TUMORS

BETA-CATENIN STATUS

QUALITY-OF-LIFE

RADIATION-THERAPY

NONMETASTATIC MEDULLOBLASTOMA

Medulloblastoma

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