Entz-Werle Natacha, Carli Emilie De, Ducassou Stéphane, Legrain Michèle, Grill Jacques, Dufour Christelle
Service de Pédiatrie, U 682 Inserm CHRU Hautepierre, Avenue Molière - 67098 Strasbourg Cedex France.
Expert Rev Anticancer Ther. 2008 Jul;8(7):1169-81. doi: 10.1586/14737140.8.7.1169.
Among pediatric malignancies, medulloblastoma (MB) is one of the most common malignant tumors of the CNS. In the past few years, thanks to a multidisciplinary approach including surgery, chemo- and radiation therapy, survival has significantly improved. Despite that, a third of patients still have a low chance of being cured and long-term survivors experience severe treatment-related sequelae. MBs are usually classified according to a clinical risk stratification, based on histological features, age at diagnosis, extent of tumor resection and presence or absence of metastases. However, these clinical variables have recently been reported to be poor for defining risk-related disease. Retrospective studies have identified histological or biological factors that have distinct roles in prognosis. As several pathways have been discovered to be involved in MB pathogenesis, they should be taken into account to more accurately stratify patients and their treatment and to develop innovative therapies.
在儿童恶性肿瘤中，髓母细胞瘤（MB）是中枢神经系统最常见的恶性肿瘤之一。在过去几年中，由于采用了包括手术、化疗和放疗在内的多学科方法，生存率有了显著提高。尽管如此，仍有三分之一的患者治愈机会较低，长期幸存者会经历严重的治疗相关后遗症。MB通常根据临床风险分层进行分类，该分层基于组织学特征、诊断时的年龄、肿瘤切除范围以及有无转移。然而，最近有报道称这些临床变量在定义风险相关疾病方面效果不佳。回顾性研究已经确定了在预后中具有不同作用的组织学或生物学因素。由于已发现多条通路参与MB的发病机制，因此应将它们考虑在内，以便更准确地对患者及其治疗进行分层，并开发创新疗法。
