尤文肉瘤家族肿瘤染色体易位及靶向治疗的研究进展  

Advances in chromosomal translocation and targeted therapy in Ewing sarcoma family of tumors

作　　者：汪红胜[1] 孙梦熊[1] 华莹奇[1] 蔡郑东[1] 

机构地区：[1]同济大学附属第十人民医院骨科,上海200072

出　　处：《肿瘤》2013年第8期748-752,共5页Tumor

摘　　要：许多骨与软组织肿瘤中存在染色体易位的现象,其中对尤文肉瘤家族肿瘤(Ewing sarcoma family of tumors,ESFT)中染色体易位的研究最早且最为深入。约85%左右的ESFT患者存在t(11;22)(q24;q12)染色体易位,由其编码的融合蛋白EWS/FLI1是导致ESFT发生和发展的关键因素。因此,对EWS/FLI1进一步研究可以为ESFT的诊断、治疗和预后等提供新的靶点。本文结合近几年的最新报道,对EWS/FLI1在ESFT发生和发展中的作用机制以及靶向治疗等方面的最新进展做一综述。Chromosomal translocation exists in various of bone and soft tissue sarcomas,especially in ESFTs （Ewing sarcoma family of tumors）.Approximately 85% of ESFTs carry a t（11;22）（q24;q12） chromosomal translocation,which generates the fusion of the 5’ segment of EWS gene with the 3’ segment of FLI 1 gene.The resultant EWS/FLI1 fusion protein promotes oncongenesis by altering the expression of its target genes,and it is necessary for the progression of ESFTs.Therefore,EWS/FLI1 has been a new target for studies on the diagnosis,treatment modality and prognosis of ESFTs.This review summarizes recent progress in research on EWS/FLI1 mechanism during the initiation and progression of ESFTs and its targeted therapy.

关 键 词：肉瘤 Ewing 易位 遗传 基因疗法 癌基因蛋白质类 融合 EWS FLI1 ESFT 

分 类 号：R738.6[医药卫生—肿瘤]