Medulloblastoma
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Medulloblastoma. / Northcott, Paul A; Robinson, Giles W; Kratz, Christian P; Mabbott, Donald J; Pomeroy, Scott L; Clifford, Steven C; Rutkowski, Stefan; Ellison, David W; Malkin, David; Taylor, Michael D; Gajjar, Amar; Pfister, Stefan M.
in: NAT REV DIS PRIMERS, Jahrgang 5, Nr. 1, 14.02.2019, S. 11.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - Medulloblastoma
AU - Northcott, Paul A
AU - Robinson, Giles W
AU - Kratz, Christian P
AU - Mabbott, Donald J
AU - Pomeroy, Scott L
AU - Clifford, Steven C
AU - Rutkowski, Stefan
AU - Ellison, David W
AU - Malkin, David
AU - Taylor, Michael D
AU - Gajjar, Amar
AU - Pfister, Stefan M
PY - 2019/2/14
Y1 - 2019/2/14
N2 - 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.
AB - 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.
KW - Chemotherapy, Adjuvant/methods
KW - Humans
KW - Magnetic Resonance Imaging/methods
KW - Mass Screening/methods
KW - Medulloblastoma/diagnosis
KW - Prognosis
KW - Quality of Life/psychology
KW - Risk Factors
U2 - 10.1038/s41572-019-0063-6
DO - 10.1038/s41572-019-0063-6
M3 - SCORING: Review article
C2 - 30765705
VL - 5
SP - 11
JO - NAT REV DIS PRIMERS
JF - NAT REV DIS PRIMERS
SN - 2056-676X
IS - 1
ER -