Department of Oncology, Division of Neuro-Oncology, St Jude Children's Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA.
Nat Rev Clin Oncol. 2014 Dec;11(12):714-22. doi: 10.1038/nrclinonc.2014.181. Epub 2014 Oct 28.
Medulloblastoma is a form of brain cancer that mainly arises during infancy and childhood. Our understanding of this disease has transitioned rapidly; what was once thought of as a single disease entity is now known to be a compendium comprising at least four distinct subtypes of tumour (Wnt, sonic hedgehog [SHH], group 3, and group 4 medulloblastomas) that have characteristic molecular signatures, distinctive clinical features, and are associated with different outcomes. Importantly, medulloblastomas occurring in infants (aged up to 3 years) and adults have unique characteristics, which distinguish the disease from that seen in children aged >3 years. Accordingly, modern treatment approaches in medulloblastoma integrate the molecular and clinical features of the disease to enable provision of the most-effective therapies for each patient, and to reduce long-term sequelae. This Review discusses our current knowledge of medulloblastoma. In particular, we present the genetic and histological features, patient demographics, prognosis, and therapeutic options for each the four molecular tumour subtypes that comprise this disease entity. In addition, the unique features of medulloblastoma in infants and in adults, as compared with childhood and/or adolescent forms, are described.
髓母细胞瘤是一种脑癌，主要发生在婴儿和儿童时期。我们对这种疾病的认识已经迅速转变；曾经被认为是单一疾病实体的疾病，现在被认为是至少包含四个不同肿瘤亚型（Wnt、sonic hedgehog [SHH]、group 3 和 group 4 髓母细胞瘤）的综合病症，这些亚型具有特征性的分子特征、独特的临床特征，并与不同的结果相关。重要的是，发生在婴儿（3 岁以下）和成人的髓母细胞瘤具有独特的特征，与 3 岁以上儿童的疾病区分开来。因此，髓母细胞瘤的现代治疗方法整合了疾病的分子和临床特征，为每个患者提供最有效的治疗方法，并减少长期后遗症。这篇综述讨论了我们对髓母细胞瘤的现有认识。特别是，我们介绍了构成该疾病实体的四个分子肿瘤亚型的遗传和组织学特征、患者人口统计学、预后和治疗选择。此外，还描述了婴儿和成人髓母细胞瘤与儿童和/或青少年形式相比的独特特征。
