儿童肾透明细胞肉瘤诊治分析

Diagnosis and treatment of clear cell sarcoma of kidney in children

目的 探讨儿童肾透明细胞肉瘤（clear cell sarcoma of the kidney,CCSK）的临床、病理特点及治疗.方法 回顾性分析2007年1月至2018年1月收治的20例CCSK患儿的临床及随访资料.其中,男11例,女9例;平均发病年龄32个月;左侧11例,右侧9例.临床表现为肉眼血尿6例,腹部包块10例,体检行腹部超声发现占位3例,腹痛1例.合并肾静脉瘤栓1例,合并腔静脉及右心房瘤栓1例.术前发现骨转移1例,肺转移并脑转移1例.结果 全部20例患儿均行瘤肾切除术,术后病理检查均提示为肾透明细胞肉瘤.按NWTS-5进行肿瘤分期：Ⅰ期11例,Ⅱ期1例,Ⅲ期6例,Ⅳ期2例.4例患儿接受术前长春新碱＋放线菌素D化疗,19例患儿接受术后长春新碱、环磷酰胺、阿霉素、依托泊苷化疗＋放疗.1例患儿因年龄小（3月龄）仅接受化疗、未接受放疗.20例患儿中,19例获访,随访时间1.5～123.0个月,平均56.1个月,存活最长的2例分别为108和85个月,6例死亡,1例失访.结论 儿童肾透明细胞肉瘤好发于3岁以下婴幼儿,易发生骨、脑转移,术前诊断困难,恶性度高、预后差,需采用手术＋化疗＋放疗的强化治疗.

Objective To explore the clinicopathological features and treatment of clear cell sarcoma of kidney （CCSK）in children.Methods The clinical and follow-up data were retrospectively analyzed for 20 CCSK children admitted from January 2007 to January 2018 .There were 11 boys and 9 girls with an average age of 32 months.The involved sides were left （n=11）and right （n=9）.The manifestations included gross hematuria （n=6）,abdominal mass （n=10）and abdominal pain （n=1）. Three cases were detected by abdominal examination.There were renal vein tumor thrombus （n=1 ）, vena cava and right atrium tumor thrombus （n=1）,preoperative bone metastases （n=1 ）and lung ＆brain metastases （n=1）.Results For 19 cases,the average follow-up period was 56.1 （1 .5-123.0） months.The longest two surviving cases were 108 and 85 months respectively.Six cases died and 1 case was follow-up.All of them underwent nephrectomy.Postoperative pathology indicated renal clear cell sarcoma.Performed according to NWTS-5,tumor stages were Ⅰ （n = 11 ）,Ⅱ （n = 1 ）,Ⅲ （n =6） and Ⅳ （n=2）.Preoperative chemotherapy （n =4）and postoperative chemotherapy plus radiotherapy （n = 19）were offered.One patient received chemotherapy alone without radiotherapy because of its young age （3 months）.Conclusions Clear cell sarcoma of kidney usually occur in infants aged under 3 years and are prone to bone and brain metastases.Preoperative diagnosis is difficult and there is high malignancy and poor prognosis.Intensive treatment of surgery plus chemotherapy plus radiotherapy is needed.