儿童恶性肾横纹肌样瘤3例临床分析及文献回顾  

Childhood malignant rhabdoid tumor of kidney: 3 cases report and review of literature

作　　者：胡慧敏[1] 张伟令[1] 黄东生[1] 张谊[1] 韩涛[1] 支天[1] 秦静[1] 

机构地区：[1]首都医科大学附属北京同仁医院儿科,北京100176

出　　处：《中国小儿血液与肿瘤杂志》2013年第6期274-276,共3页Journal of China Pediatric Blood and Cancer

摘　　要：目的通过总结3例原发于肾脏的儿童恶性横纹肌样瘤的临床资料,探讨儿童肾横纹肌样瘤的诊断、治疗及预后。方法收集我院近8年来收治的3例病理明确诊断的原发于肾脏的恶性横纹肌样瘤(男2例,女1例),对其发病特点、病理特点、治疗及随访等临床资料进行总结分析,并复习相关文献。结果根据国际儿童肿瘤组织肾横纹肌样瘤的临床分期诊断,3例患儿均为Ⅲ期。均行手术、化疗及放疗治疗。化疗以ICE与VDC交替方案为主,化疗周期分别为6、6、15周期,放疗采用外放疗。随访至2012年7月(3例随访时间分别是8、14、8个月)。2例获完全缓解(CR),1例复发后经手术、化疗及放疗后病情部分缓解(PR)。结论恶性肾横纹肌样瘤是一种少见、病理形态独特的肿瘤。治疗应在明确诊断和确切分期的基础上,行手术、化疗和选择性放疗的综合性治疗。Objective To summarize the clinical data of three children with malignant rhahdoid tumor of kidney （MRTK）, we discuss the diagnosis, treatment and prognosis of the disease. Methods We collected the clinical data of 3 cases who were diagnosed as MRTK by pathology, 2 of them were boys. The clinical and pathological features of these patients were analyzed, and the related literatures were reviewed. Results In accordance with International Children＇ s Organizations of Rhabdoid Tumor of Kidney ,the three patients＇ clinical stages were stage Ill. They were given the comprehensive treatment including operation, radiotherapy and chemotherapy. The chemotherapy protocols were ICE and VDC alternately. The chemotherapy treatment cycles were 6, 6 and 15 respectively, combined with external radiotherapy. Until to July 2012 two patients were complete remission （CR） and one patient was partial remission （PR） after given operation, chemotherapy and radiotherapy in spite of recurrence. The follow- up time was separately 8 m, 14m and 8m. Conclusion Malignant rhabdoid tumor of kidney is one kind of tumor which is seldom seen, the pathological features of which is unique. Combined treatment should be given based on clear diagnosis and precise stage. The combined treatments included surgery, chemotherapy and selective radiation therapy.

关 键 词：横纹肌样瘤 病理学 诊断 治疗 儿童 

分 类 号：R737.11[医药卫生—肿瘤]