中文摘要：

目的：探讨儿童肾脏恶性横纹肌样瘤（malignant rhabdoid tumor of the kidney，MRTK）的临床及病理特点。方法回顾性分析2009年1月至2015年4月本院收治的15例 MRTK 患儿临床、病理及随访资料。结果15例患儿中，男性7例，女性8例，年龄3～28个月，平均年龄12．6个月。左侧6例，右侧9例。血尿10例，腹部包块4例，超声发现1例。按 NWTS 3进行肿瘤分期：Ⅰ期2例，Ⅱ期5例，Ⅲ期5例，Ⅳ期3例。术前予化疗1例，肾动脉栓塞1例。予保留肾单位的肿瘤剜除术1例，瘤肾切除14例。病理检查结果均提示肾脏恶性横纹肌样瘤。术后化疗14例，放疗4例。11例获随访，9例死亡，2例无瘤存活分别2年、6年，4例失访。结论儿童肾脏恶性横纹肌样瘤好发于婴幼儿，血尿多见，进展迅速，恶性度高，易发生肺脑转移，需要手术＋化疗＋放疗的综合治疗，预后差。

英文摘要：

Objetive To explore the clinicohistological features of malignant rhabdoid tumor of the kid-ney （MRTK）. Methods Retrospective reviews and biological analysis of MRTK were performed. Results Fifteen MRTK children （7 boys,8 girls）with a median age of 12.6 （3 ~28）months were recruited.There were hematuria （n =10）,abdominal mass （n =4）and ultrasonic finding （n =1）.According to the National Wilms′Tumor Study 3 staging scheme,the stages were Ⅰ （n =2）,Ⅱ （n =5）,Ⅲ（n =5）and Ⅳ （n =3） .Nephrectomy was performed for affected kidney in 14 patients while one case underwent nephron-sparing sur-gery.Postoperative pathology confirmed MRTK in all 15 cases.They received postoperative chemotherapy and 4 patients had radiotherapy.Follow -ups were available for 11 cases.Nine patients died while 2 cases survived for 2 ~6 years. Conclusions MRTK is a rare,highly aggressive and malignant tumor predominantly in in-fants and young children.Hematuria is a common symptom.Most MRTK patients have advanced stage disease so that the outcomes of chemoradiotherapy remain unsatisfactory.