儿童肾透明细胞肉瘤的临床分析

  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。 

Clinical analysis of clear cell sarcoma of the kidney in children

  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 2009and December 2014 were retrospectively analyzed.Follow-ups were conducted to monitor the postoperative conditions of the patients.  Results  Nine cases of CCSK were recruited, and another three cases were excluded for the analysis because of incomplete clinical data.All patients achieved complete remission when the treatment was finished.The estimated 3-year overall survival rate was100%, and the estimated 3-year event-free survival rate was 83.3%.No report is available on the significant side effects associatedwith this treatment.  Conclusion  CCSK is rare in children and is easily misdiagnosed.The chemotherapy based on the WT-2009 protocol can produce a favorable prognosis and a high tolerance for patients with CCSK.However, the treatment for high-risk patient needsto be further explored, and follow-ups must be intensified.