摘要
肾母细胞瘤是最常见的儿童原发性泌尿系统恶性肿瘤,具有早期诊断难、预后欠佳、易复发等特点。研究表明具有肾母细胞瘤倾向的综合征、肾母细胞瘤发病机制相关的基因通路(WT1、WTX、TP53、MYCN及基因拷贝数变异等)、表观遗传机制(印记基因、甲基化)、Micro RNAs、DNA错配修复系统等多种因素均参与肾母细胞瘤的发生发展,但一些结论仍然需要大量基础和临床研究来支持。肾母细胞瘤发病机制纷繁复杂,应充分利用现有研究进展,开展遗传咨询及个体化治疗。全文就近年来国内外对于肾母细胞瘤发病机制研究的最新进展作一系统综述。
Wilms tumor is the most common malignant tumor of urinary tract in children.It is difficult for early diagnosis,likely to relapse and the prognosis is poor. Studies show that various factors are involved in the occurrence and development of Wilms tumor,including the gene pathways（WT1,WTX,TP53,MYCN and gene copy number alterations）,the epigenetic mechanism（imprinting genes and methylation）,Micro RNAs,DNA mismatch repair system and so on. However,it still needs further studies to support these findings.The pathogenesis of Wilms tumor is complicated,so it is necessary to carry out genetic counseling and individualized treatment based on the current research progress. This article reviews the latest research progress on the pathogenesis of Wilms tumor.
作者
曾蕊
常会波
吴建新
ZENG Rui CHANG Hui-bo WU Jian-xin(Capital Institute of Pediatrics, Beijing 100020, China)
出处
《中国肿瘤》
CAS
CSCD
2017年第6期452-459,共8页
China Cancer
基金
国家自然科学基金青年项目(81301773)
