中晚期儿童肾透明细胞肉瘤10例的诊治及预后分析  

Diagnosis,treatment and prognosis of 10 children with advanced clear cell sarcoma of kidney

作　　者：胡慧敏[1] 张伟令[1] 黄东生[1] 王一卓[1] 张谊[1] 易优[1] 李静[1] 周燕[1] Hu Huimin;Zhang Weiling;Huang Dongsheng;Wang Yizhuo;Zhang Yi;Yi You;Li Jing;Zhou Yan(Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University,Beijing 100176,China)

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：《中华实用儿科临床杂志》2020年第5期370-374,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：北京市医院管理局"登峰"人才培养计划(DFL20180201)。

摘　　要：目的探讨中晚期儿童肾透明细胞肉瘤(CCSK)的临床特点、诊治经过及预后特点。方法收集2014年1月至2017年12月在首都医科大学附属北京同仁医院儿科住院治疗的10例中晚期CCSK患儿的临床资料,对其临床特征、诊疗经过及预后情况等进行回顾性分析。结果1.临床特点:10例CCSK病例中男6例,女4例;中位发病年龄为32个月;7例为左侧CCSK,3例为右侧CCSK。初次诊断时Ⅲ期9例,Ⅳ期1例;其中4例初次诊断时误诊为肾脏其他肿瘤(40%,4/10例)。5例Ⅲ期病例治疗及随访过程中出现肿瘤复发及转移,主要远处转移部位为肺、骨、肝脏及脑。2.治疗及预后:10例中给予手术联合放疗及化疗者7例,未规范化治疗放弃者3例。中位随访时间33.5个月,7例存活,3例死亡,3年总生存率为65.6%。Ⅲ期患儿3年总生存率为74.1%,Ⅳ期患儿3年总生存率为0,Ⅲ期与Ⅳ期预后比较差异有统计学意义(χ^2=9,P=0.003)。5例复发病例中仅1例完全缓解,2例部分缓解,1例进展,1例死亡;3例无复发病例均完全缓解,且均为给予手术、化疗及放疗规范化治疗者。结论儿童CCSK初诊误诊率高,Ⅲ期病例治疗及随访过程中复发及远处转移风险高;Ⅲ期病例积极给予手术、化疗及放疗的规范化治疗,预后良好,而发生复发及远处转移者死亡率高。Objective To investigate the clinical features,diagnosis,treatment and prognosis of advanced clear cell sarcoma of kidney(CCSK)in children.Methods The clinical data of 10 children with advanced CCSK hospitalized in Department of Pediatrics of Beijing Tongren Hospital,Capital Medical University from January 2014 to December 2017 were collected,and their clinical features,diagnosis,treatment and prognosis were analyzed retrospectively.Results(1)Clinical features of CCSK:ten cases of CCSK included 6 boys and 4 girls,with the median onset age of 32 months;7 cases were left CCSK and 3 cases were right CCSK.There were 9 cases of stageⅢand 1 case of stageⅣat the time of initial diagnosis,when 4 cases were misdiagnosed as other renal tumors at the time of initial diagnosis(40%,4/10 cases).Five patients with stageⅢCCSK had recurrence and metastasis during treatment and follow-up,and the main distant metastasis sites were lung,bone,liver and brain.(2)Treatment and prognosis of CCSK:seven cases received surgery combined with radiotherapy and chemotherapy,and 3 cases whose parents gave up treatment adopted non-standardized treatment.The median follow-up time was 33.5 months.Seven patients survived and 3 cases died.The 3-year overall survival rate of all 10 patients was 65.6%.The 3-year overall survival rate of stageⅢwas 74.1%,and that of stageⅣwas 0.The prognosis of stageⅢwas significantly better than that of stageⅣ(χ^2=9,P=0.003).Among the 5 recurrent cases,only 1 case achieved completely remission,2 cases achieved partially remission,1 case suffered from disease progression and 1 case died.The 3 cases without recurrence were given standardized treatment of surgery,che-motherapy and radiotherapy,and all were completely remitted.Conclusions CCSK is easy to be misdiagnosed,and the risk of recurrence and distant metastasis is high in stageⅢpatients during treatment and follow-up.StageⅢpatients who actively receive standard treatment including surgery,chemotherapy and radiotherapy have good prognosis,while th

关 键 词：肾透明细胞肉瘤 儿童 诊断 预后 

分 类 号：R737[医药卫生—肿瘤]