The Arthur and Sonia Labatt Brain Tumor Research Centre, Division of Neurosurgery, The Hospital for Sick Children, the University of Toronto, Suite 1503, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Department of Neurosurgery, Cantonal Hospital of Aarau, Aarau, Switzerland.
Curr Neurol Neurosci Rep. 2016 May;16(5):43. doi: 10.1007/s11910-016-0644-7.
Medulloblastoma is a malignant embryonic brain tumor arising in the posterior fossa and typically occurring in pediatric patients. Current multimodal treatment regimes have significantly improved the survival rates; however, a marked heterogeneity in therapy response is observed, and one third of all patients die within 5 years after diagnosis. Large-scale genetic and transcriptome analysis revealed four medulloblastoma subgroups (WNT, SHH, Group 3, and Group 4) associated with different demographic parameters, tumor manifestation, and clinical behavior. Future treatment protocols will integrate molecular classification schemes to evaluate subgroup-specific intensification or de-escalation of adjuvant therapies aimed to increase tumor control and reduce iatrogenic induced morbidity. Furthermore, the identification of genetic drivers allows assessing target therapies in order to increase the chemotherapeutic armamentarium. This review highlights the biology behind the current classification system and elucidates relevant aspects of the disease influencing forthcoming clinical trials.
髓母细胞瘤是一种起源于后颅窝的恶性胚胎脑肿瘤，通常发生在儿科患者中。目前的多模式治疗方案显著提高了生存率；然而，治疗反应存在明显的异质性，所有患者中有三分之一在诊断后 5 年内死亡。大规模的遗传和转录组分析揭示了与不同人口统计学参数、肿瘤表现和临床行为相关的四个髓母细胞瘤亚组（WNT、SHH、第 3 组和第 4 组）。未来的治疗方案将整合分子分类方案，以评估辅助治疗的亚组特异性强化或降级，旨在提高肿瘤控制率并降低医源性发病率。此外，遗传驱动因素的鉴定允许评估靶向治疗，以增加化疗武器库。本综述强调了当前分类系统背后的生物学，并阐明了影响未来临床试验的疾病相关方面。
