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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。
Objective To investigate the clinical and pathological features of malignant rhabdoid tumor of the kidney (MRTK) in children. Methods The clinical, pathological and follow-up data of 15 MRTK children admitted to our hospital from January 2009 to April 2015 were retrospectively analyzed. Results Of the 15 children, 7 were male and 8 were female, ranging in age from 3 to 28 months with a mean age of 12.6 months. 6 cases on the left and 9 cases on the right. Hematuria in 10 cases, abdominal mass in 4 cases, ultrasound in 1 case. According to NWTS 3 tumor staging: Ⅰ in 2 cases, Ⅱ in 5 cases, Ⅲ in 5 cases, Ⅳ in 3 cases. Preoperative chemotherapy in 1 case, renal artery embolization in 1 case. To retain the nephron spondylosis in 1 case, nephrectomy in 14 cases. Pathological examination showed renal malignant rhabdomyosarcoma. Postoperative chemotherapy in 14 cases, 4 cases of radiotherapy. Eleven patients were followed up, 9 died and 2 survived 2 years and 6 years, respectively. Four patients were lost to follow-up. Conclusions Malignant rhabdomyosarcoma in children is common in infants and children with hematuria. Malignant rhabdomyosarcoma is more common in children and adolescents. The progress is rapid, the malignant degree is high, and the lung and brain metastasis are easy to occur. The comprehensive treatment of operation + chemotherapy + radiotherapy is needed, and the prognosis is poor.