儿童肾透明细胞肉瘤的多学科诊治与随访

Multidisciplinary diagnosis, treatment, and follow-up of children with clear cell sarcoma of kidney

目的·探讨儿童肾透明细胞肉瘤的临床特点与多学科联合治疗。方法·收集2011年1月—2016年1月上海交通大学医学院附属新华医院收治的7例肾透明细胞肉瘤患儿的资料,对临床表现、影像学和病理学特点及治疗和随访情况等进行回顾性分析。结果·7例患儿中,男6例,女1例;中位年龄为28个月(4~59个月);I期3例,Ⅲ期2例,Ⅳ期2例。经小儿血液/肿瘤科、儿外科、病理科、放射科与放疗科等多学科讨论和评估后,纳入CCCG-WT-2009方案治疗。中位随访时间为22个月(8~56)个月;6例存活,1例死亡。结论·多学科联合诊治模式能有效改善儿童肾透明细胞肉瘤的预后,CCCG-WT-2009方案对早期儿童肾透明细胞肉瘤的治疗效果好,治愈率高;晚期儿童肾透明细胞肉瘤的治疗有待进一步探讨和完善。

To investigate the clinical characteristics and multidisciplinary treatment of children with clear cell sarcoma of kidney (CCSK).Methods · Data of seven children with CCSK treated at Xinhua Hospital, Shanghai Jiao Tong University School of Medicine between Jan 2011 and Jan 2016 were collected. The retrospective analysis of clinical manifestations, features of imaging and pathology, treatment, and follow-up was performed.Results · Of 7 children with CCSK, 6 were male and 1 was female with the median age of 28 months (4-59 months), and 3 were at stage Ⅰ , 2 at stage Ⅲ,and 2 at stage Ⅳ . All cases were discussed and evaluated by multidisciplinary teams, including pediatric hematology/oncology, pediatric surgery, pathology,radiology and radiotherapy. CCCG-WT-2009 protocol was adopted to treat these patients. The median follow-up period was 22 months (8-56 months).Six children survived and one died. Conclusion · The multidisciplinary treatment mode can effectively improve the prognosis of CCSK. CCCG-WT-2009protocol has good therapeutic effect and high cure rate for children with early stage CCSK, but the treatment of advanced stage CCSK needs to be further explored and perfected.