儿童肾透明细胞肉瘤的临床分析

Clinical analysis of clear cell sarcoma of the kidney in children

目的:探讨儿童肾透明细胞肉瘤的临床特点及肾脏肿瘤治疗方案(WT-2009方案)治疗该病的疗效和安全性。方法:收集天津医科大学肿瘤医院2009年1月至2014年12月收治的肾透明细胞肉瘤患儿病例12例,因临床数据不完整等原因剔除3例,共计9例资料,对其临床表现和治疗过程以及随访结果进行回顾性分析。结果:经综合治疗后9例患儿均完全缓解(100%),随访满3年的6例患儿,3年总生存率100%,3年无事件生存率83.3%,未见明显治疗相关不良反应。结论:肾透明细胞肉瘤发病率低,误诊率高,WT-2009治疗方案近期疗效好,高危患者的治疗方案有待完善,远期治疗效果需进一步随访。

Objective： To summarize the clinical features of clear cell sarcoma of the kidney （CCSK） in children and to evaluate the efficacy and safety of the WT-2009 chemotherapy protocol. Methods： Clinical data of children with CCSK recorded between January 2009 and December 2014 were retrospectively analyzed. Follow-ups were conducted to monitor the postoperative conditions of the pa- tients. Results： Nine cases of CCSK were recruited, and another three cases were excluded for the analysis because of incomplete clini- cal data. All patients achieved complete remission when the treatment was finished. The estimated 3-year overall survival rate was 100%, and the estimated 3-year event-free survival rate was 83.3%. No report is available on the significant side effects associated with this treatment. Conclusion： CCSK is rare in children and is easily misdiagnosed. The chemotherapy based on the WT-2009 proto- col can produce a favorable prognosis and a high tolerance for patients with CCSK. However, the treatment for high-risk patient needs to be further explored, and follow-ups must be intensified.