儿童肾脏恶性横纹肌样瘤临床诊治经验分析  

Diagnosis and treatment of malignant rhabdoid tumor of the kidney in children

作　　者：潘俊涛[1] 张大[1] 宋东健 崔西春[1] 范应中[1] 王家祥[1] PAN Juntao;ZHANG Da;SONG Dongjian;CUI Xichun;FAN Yingzhong;WANG Jiaxiang(Department of Pediatric Surgery,the First Affiliated Hospital,Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院小儿外科,河南郑州450052

出　　处：《肿瘤基础与临床》2020年第1期51-54,共4页journal of basic and clinical oncology

基　　金：国家自然科学基金青年基金资助项目(81902471)。

摘　　要：目的总结儿童肾脏恶性横纹肌样瘤的临床特点及诊治经验。方法对郑州大学第一附属医院2014年1月至2019年10月收治的肾脏恶性横纹肌样瘤患儿进行回顾性分析并随访。结果共入组肾脏恶性横纹肌样瘤患儿15例,男8例,女7例,年龄3~19个月,中位发病年龄9个月。左侧5例,右侧10例。肉眼血尿10例(外伤后肉眼血尿1例),腹部包块3例,发热1例,体检超声发现1例。术前发现肺转移1例,予以诱导化疗。15例肾脏恶性横纹肌样瘤患儿全部行带瘤肾脏切除术,病理检查结果均提示肾脏恶性横纹肌样瘤。按美国肾母细胞瘤协作组-5临床病理分期标准进行肿瘤分期Ⅰ期8例,Ⅱ期2例,Ⅲ期4例,Ⅳ期1例。14例行一期肿瘤切除术;1例予以诱导化疗后未见瘤灶缩小,行延期肿瘤切除术。术后化疗14例,均未放疗。15例患儿均获得随访,除1例19个月患儿术后至今(11个月)仍未见肿瘤复发且存活外,其余14例均在术后2~9个月死亡,均为肿瘤快速复发进展所致。结论儿童肾脏恶性横纹肌样瘤好发于1岁以下婴幼儿,临床以肉眼血尿最多见,进展迅速,恶性度高,易发生肺转移,确诊有赖于病理检查,争取手术全切除结合术后放、化疗是目前主要治疗方法,总体预后差。Objective To summarize the clinical characteristics and surgical strategy of malignant rhabdoid tumor of the kidney in children.Methods A retrospective analysis was performed in the 15 patients with malignant rhabdoid tumor of the kidney from 2014 Jan to 2019 Oct in the First Affiliated Hospital of Zhengzhou University.Results Fifteen malignant rhabdoid tumor of the kidney children(8 boys,7 girls)with the median age of 9(3-19)months were recruited.There were 5 cases in the left kidney and 10 cases in the right kidney.There were 10 cases of hematuria,3 cases of abdominal mass,1 case of fever and 1 case of altrasonic finding.One case of lung metastasis was found before operation,and the chemotherapy was induced.Nephrectomy was performed for the affected kidney in all the 15 patients,and the postoperative pathology conformed malignant rhabdoid tumor of the kidney.According to the National Wilms Tumor Study-5 staging method,stageⅠwas in the 8 cases,stageⅡin the 2 cases,stageⅢin the 4 cases,stageⅣin the 1 case.Upfront surgery was performed in the 14 cases,and 1 case of stageⅣpatient received the delayed resection after induction chemotherapy.Fourteen cases received post operation chemotherapy without radiotherapy.Follow-ups were available for 15 cases.Except that 1 case of 19 month old child who received chemical therapy after surgical total resection was still alive,the other 14 children died of tumor recurrence and progression at 2 to 9 months after operation.Conclusion malignant rhabdoid tumor of the kidney in children is a rare highly aggressive and malignant tumor that occur predominantly in infants younger than 1 year,whose diagnosis depends on the pathological findings.The gross hematuria,rapid progress,high malignancy and lung metastasis are their characteristics.The diagnosis depends on pathology.Total resection combined with radiochemotherapy is the main treatment,but the prognosis is poor.

关 键 词：恶性横纹肌样瘤 肾脏 儿童 

分 类 号：R737.11[医药卫生—肿瘤]