髓母细胞瘤生存预后相关因素的分子生物学研究进展

Current Progress on Study of Molecular Biology Relating to Prognosis of Medulloblastoma

髓母细胞瘤(medulloblastoma,MB)属高侵袭性的原始外胚层肿瘤(primitive neuroectodermal tumors,PNET),目前的治疗方法包括手术及放化疗,对于年幼患儿的疗效仍不满意。小脑半球的形成调节通路研究对肿瘤的发生在分子及生物水平做出一定的解释。多项研究证实Shh(sonic hedgehog)和Wnt通路在其中具有重要作用。未来的MB肿瘤分期及预后将包括某些分子标志物,寻找髓母细胞瘤中关键的分子标志物,可对髓母细胞瘤进行更为特异性的分级,判断患者预后。建立一套更精确的分期系统可更好区别高风险与普通风险MB,寻找最有效的治疗靶向,从而找到最佳治疗策略。

Medulloblastomas（MBs） are highly aggressive primitive neuroectodermal tumors（PNET） usually located in the posterior fossa.Current treatment for MBs,which includes a combination of surgery,chemotherapy and radiation,remain challenging especially in younger patients.However,advances in the understanding of regulatory pathways in cerebellar development have elucidated possible areas of dysfunction involved in tumorigenesis.Multiple studies have demonstrated the importance of the sonic hedgehog,Wnt,and Notch pathways in MB pathogenesis at the molecular level.While staging and prognosis are often based on the Chang classification system,future algorithms will involve identifying molecular markers in order to allow for more specific risk stratifications of various MB subtypes and provide improved correlation with staging and prognosis.Future development of novel therapies thattarget the heterogeneity of MB and are tailored to the tumor’s unique molecular profile may yield improved outcomes for these patients.