Franceschi Enrico, Giannini Caterina, Furtner Julia, Pajtler Kristian W, Asioli Sofia, Guzman Raphael, Seidel Clemens, Gatto Lidia, Hau Peter
Nervous System Medical Oncology Department, IRCCS Istituto delle Scienze Neurologiche di Bologna, Via Altura 3, 40139 Bologna, Italy.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 59005, USA.
Cancers (Basel). 2022 Jul 29;14(15):3708. doi: 10.3390/cancers14153708.
Medulloblastoma (MB) is a malignant embryonal tumor of the posterior fossa belonging to the family of primitive neuro-ectodermic tumors (PNET). MB generally occurs in pediatric age, but in 14-30% of cases, it affects the adults, mostly below the age of 40, with an incidence of 0.6 per million per year, representing about 0.4-1% of tumors of the nervous system in adults. Unlike pediatric MB, robust prospective trials are scarce for the post-puberal population, due to the low incidence of MB in adolescent and young adults. Thus, current MB treatments for older patients are largely extrapolated from the pediatric experience, but the transferability and applicability of these paradigms to adults remain an open question. Adult MB is distinct from MB in children from a molecular and clinical perspective. Here, we review the management of adult MB, reporting the recent published literature focusing on the effectiveness of upfront chemotherapy, the development of targeted therapies, and the potential role of a reduced dose of radiotherapy in treating this disease.
髓母细胞瘤（MB）是后颅窝的一种恶性胚胎性肿瘤，属于原始神经外胚层肿瘤（PNET）家族。MB通常发生于儿童期，但在14% - 30%的病例中，它会影响成年人，大多数为40岁以下，每年发病率为百万分之0.6，约占成人神经系统肿瘤的0.4% - 1%。与儿童MB不同，由于青少年和年轻成年人中MB发病率较低，针对青春期后人群的有力前瞻性试验很少。因此，目前老年患者的MB治疗很大程度上是从儿童经验推断而来，但这些治疗模式对成年人的可转移性和适用性仍是一个悬而未决的问题。从分子和临床角度来看，成人MB与儿童MB不同。在此，我们回顾成人MB的治疗，报告近期发表的文献，重点关注初始化疗的有效性、靶向治疗的发展以及降低放疗剂量在治疗该疾病中的潜在作用。
