MD Anderson Cancer Center Department of Neuro-oncology, 1515 Holcombe Blvd., Houston, TX, 77030, USA.
NCI/NOB, NIH, Bloch Bldg. 82, Room 213, 9030 Old Georgetown Rd., Bethesda, MD, 20892, USA.
Curr Treat Options Oncol. 2019 Jun 24;20(8):64. doi: 10.1007/s11864-019-0663-0.
Medulloblastoma (MB) is a malignant embryonal tumor of the posterior fossa and is the most common type of brain cancer in pediatric patients. In contrast, adult MB is very rare with an incidence of 0.6 per million per year and mostly affects young adults below the age of 40. Recent molecular analyses of pediatric and adult MB have classified these tumors into at least four individual molecular subgroups (SHH, WNT, group 3, and group 4) with distinct demographics, histology, and prognosis. The discrete biological composition of these tumors likely explains the marked heterogeneity in responses seen to conventional therapies such as radiation and cytotoxic chemotherapies. Given the low incidence of adult MB, prospective studies are challenging and scarce, and management guidelines are largely derived from the pediatric MB patient population and retrospective data. However, adult MB is clinically and molecularly distinct from pediatric MB and a comprehensive review of published literature on adult MB highlighting their differences is warranted. Here, we review the management of adult MB focusing on recent studies exploring the effectiveness of upfront chemotherapy, clinical trials in the context of molecular subgroup-specific therapies, and the potential role of immunotherapy in treating this disease.
髓母细胞瘤（MB）是一种后颅窝的恶性胚胎肿瘤，是儿科患者中最常见的脑癌类型。相比之下，成人 MB 非常罕见，发病率为每年每百万分之 0.6，主要影响 40 岁以下的年轻成年人。最近对儿科和成人 MB 的分子分析将这些肿瘤分为至少四个单独的分子亚组（SHH、WNT、第 3 组和第 4 组），具有不同的人口统计学、组织学和预后。这些肿瘤的离散生物学组成可能解释了对常规治疗（如放疗和细胞毒性化疗）的反应存在明显异质性。鉴于成人 MB 的发病率较低，前瞻性研究具有挑战性且稀缺，管理指南主要来自儿科 MB 患者人群和回顾性数据。然而，成人 MB 在临床和分子上与儿科 MB 不同，因此有必要对已发表的成人 MB 文献进行全面审查，强调它们的差异。在这里，我们回顾了成人 MB 的治疗方法，重点介绍了探索 upfront 化疗效果的最新研究、分子亚组特异性治疗背景下的临床试验以及免疫疗法在治疗这种疾病中的潜在作用。
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