Childhood medulloblastoma: novel approaches to the classification of a heterogeneous disease

被引:83
作者
Ellison, David W. [1 ]
机构
[1] St Jude Childrens Res Hosp, Dept Pathol MS 250, Memphis, TN 38103 USA
关键词
Medulloblastoma; Stratification; Classification; Molecular pathology; Shh; Wnt; PRIMITIVE NEUROECTODERMAL TUMORS; COMPARATIVE GENOMIC HYBRIDIZATION; CRANIOSPINAL RADIATION-THERAPY; HEDGEHOG PATHWAY INHIBITOR; IN-SITU HYBRIDIZATION; BETA-CATENIN STATUS; POSTOPERATIVE CHEMOTHERAPY; ADJUVANT CHEMOTHERAPY; WNT/WINGLESS PATHWAY; ISOCHROMOSOME; 17Q;
D O I
10.1007/s00401-010-0726-6
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Increasing recognition of pediatric medulloblastoma as a heterogeneous disease, with histopathological and molecular variants that have distinct biological behaviors, will impact how the disease is classified and treated. A combination of clinicopathological evaluation and assays based on molecular subgroups of disease will allow stratification of patients into risk groups and a more tailored approach to therapy. Patients with low-risk disease could be treated with de-escalated adjuvant therapy to maximize cure while reducing long-term adverse effects, and novel therapies could be sought for patients with high-risk disease. My review encompasses a brief overview of the clinical landscape, the current World Health Organization (WHO) classification of medulloblastoma, the status of molecular subgroups, and how potential stratification schemes might impact pathologists and their practice.
引用
收藏
页码:305 / 316
页数:12
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