儿童肾透明细胞肉瘤5例报告

Report of 5 cases of renal clear cell sarcoma in children

目的总结5例儿童肾透明细胞肉瘤(CCSK)诊治过程及预后,探讨其临床特征及诊治方向。方法回顾性分析经我院诊治的5例儿童肾透明细胞肉瘤患者的临床资料并结合文献探讨其诊疗过程。患者平均年龄21.6(12～38)月;男4例,女1例。1例体检发现入院,3例发现腹部巨大肿块入院,1例腹部肿块伴肉眼血尿入院。5例肿瘤均为单侧:左2例,右3例;转移2例(骨转移1例,骨、肺转移1例。)均行开放肾根治性切除术,术后给予化疗。结果手术均顺利进行,切除范围包括瘤体及残余肾组织,无并发症。术后病理证实为肾透明细胞肉瘤。随访患者均正常存活,未见原位复发案例。结论根治性肾肿瘤切除术联合化疗的肾透明细胞肉瘤患儿可获得较长无瘤生存期。早发现、手术联合化疗可改善CCSK患儿预后。

Objective To explore the diagnosis,treatment,clinical features and prognosis of renal clear cell sarcoma in children.Methods The clinical data,imaging and pathological results of 5 children with renal clear cell sarcoma treated in our hospital were retrospectively analyzed and related literature was reviewed.The patients were 4 boys and 1 girl,average age 21.6 months(12-38).Of all patients,1 was admitted after physical examination,1 due to abdominal mass and hematuria,and 3 due to massive abdominal mass.All tumors were unilateral,including 2 in the left,and 3 in the right.Metastasis occurred in 2 patients,including bone metastasis in 1 case,and bone and lung metastasis in 1 case.All patients underwent open radical nephrectomy and postoperative chemotherapy.Results All operations were successful.The resection range included the tumor and residual renal tissues.No complications were observed.Postoperative pathology confirmed renal cell sarcoma.During the followup,all patients survived and no recurrence occurred.Conclusion Patients with renal cell sarcoma can obtain a relatively long non-tumor survival with radical nephrectomy combined with postoperative chemotherapy.Early detection,surgery and chemotherapy can improve the prognosis.