髓母细胞瘤的研究新视角  

A New Perspective for the Study of Medulloblastoma

作　　者：何密斯[1] 李昱[2] 唐俐[1] 

机构地区：[1]重庆医科大学病理生理学教研室,重庆400016 [2]重庆医科大学病理学教研室,重庆400016

出　　处：《中国神经肿瘤杂志》2013年第1期47-52,共6页Chinese Journal of Neuro-Oncology

基　　金：国家自然科学基金(No.81272571);重庆市自然科学基金(No.2009BB5064)

摘　　要：髓母细胞瘤(medulloblastoma,MB)是一种高度恶性的中枢神经系统肿瘤,好发于儿童,预后极差。MB的标准的治疗方案包括手术切除和/或联合放化疗,患者生存率有所提高,但放化疗后遗症大大降低了存活者的生活质量。近年来,分子生物学研究技术快速发展,基于基因组模式的分型方式对MB有了全新的认识视角。目前普遍认为MB可分为4个独立的分子类型,即WNT型(WNT subtype)、SHH型(SHH subtype)、3型(Group 3)和4型(Group 4)。各个亚型之间在临床特征、病理分型、分子生物学、预后等方面均具有显著区别。深化MB分子生物学研究,探索各亚型特有的发病机制,优化危险分层标准及个体化治疗方案,以期找到新的高效、低毒副作用的药物,提高患者生存率、减低治疗后遗症将成为MB研究的新焦点。Medulloblastoma （MB） is the most common malignant childhood brain tumour and the prognosis is poor. Currently available therapy for patients consists of maximal safe resection, followed by radiation therapy and chemotherapy. Although survival rate increases, survivors experience intellectual and neurological disabilities secondary to both disease and therapy. Recent technological advances in molecular biology has uncovered that medulloblastoma consists of four distinct molecular subgroups： WNT, SHH, Group 3 and Group 4. Medulloblastoma subgroups have highly disparate cytogenetics, mutational spectra and gene expression signatures, in addition to divergent clinical phenotypes such as patient demographics, tumour cell histology and patient outcome. To study the four main subgroups of medulloblastoma may enable appropriate therapeutic stratification and use of patient-specific, targeted anti-cancer agents to improve clinical care of patients.

关 键 词：髓母细胞瘤 基因组学 分子分型 

分 类 号：R739.41[医药卫生—肿瘤]