Vigneron C, Entz-Werlé N, Lutz P, Spiegel A, Jannier S, Helfre S, Alapetite C, Coca A, Kehrli P, Noël G
Département de radiothérapie, centre de lutte contre le cancer Paul-Strauss, 3, rue de la Porte-de-l'Hôpital, BP 42, 67065 Strasbourg cedex, France.
Service d'oncologie pédiatrique, CHU Hautepierre, 1, avenue Molière, 67098 Strasbourg cedex, France.
Cancer Radiother. 2015 Aug;19(5):347-57; quiz 358-9, 362. doi: 10.1016/j.canrad.2015.03.010. Epub 2015 Jul 2.
Medulloblastoma are cerebellar tumours belonging to the group of primitive neuroectodermal tumours (PNET) and are the most common malignant brain tumours of childhood. These tumours are rare and heterogeneous, requiring some multicentric prospective studies and multidisciplinary care. The classical therapeutic approaches are based on clinical, radiological and surgical data. They involve surgery, radiation therapy and chemotherapy. Some histological features were added to characterize risk. More recently, molecular knowledge has allowed to devise risk-adapted strategies and helped to define groups with good outcome and reduce long-term sequelae, improve the prognostic of high-risk medulloblastoma and develop new therapeutic tools.
髓母细胞瘤是属于原始神经外胚层肿瘤(PNET)组的小脑肿瘤,是儿童期最常见的恶性脑肿瘤。这些肿瘤罕见且具有异质性,需要一些多中心前瞻性研究和多学科护理。经典的治疗方法基于临床、放射学和手术数据。它们包括手术、放射治疗和化疗。增加了一些组织学特征来表征风险。最近,分子知识使得能够制定风险适应性策略,并有助于定义预后良好的群体,减少长期后遗症,改善高危髓母细胞瘤的预后,并开发新的治疗工具。