儿童肾细胞癌(RCC)的临床病理特征及手术疗效分析

 目的 探讨儿童肾细胞癌（renal cell carcinoma，RCC）的发病特点、临床表现、病理学特征及手术治疗方式和疗效。方法 回顾性分析复旦大学附属儿科医院2008年8月至2018年8月收治的21例儿童RCC病例的临床资料。年龄：1.2~10.8岁，中位年龄6.1岁；性别：男14例，女7例；发病部位：左肾13例，右肾8例。患儿中6例以无痛肉眼血尿、3例以腹痛、2例以腹部肿块为首发症状就诊，其余10例由健康检查彩超发现。结果 21例患儿均接受手术治疗。14例行根治性肾切除术，其中6例同时行腹膜后淋巴结清扫术；5例行保留肾单位肾肿瘤切除术（nephron-sparing surgery，NSS），其中4例肿瘤最大径<4 cm，1例肿瘤最大径7.8 cm；2例仅行肾肿瘤活检术。术后病理分类为：RCC 9例，囊性RCC 1例，Xp11.2易位/TFE3基因融合相关性RCC 11例。TNM分期为：T1aN0M0/Ⅰ期7例，T1bN0M0/Ⅰ期3例，T1aN1M0/Ⅲ期1例，T1bN1M0/Ⅲ期1例，T2aN0M0/Ⅱ期3例，T2aN1M0/Ⅱ期1例，T2bN1M0/Ⅲ期1例，T3aN0M0/Ⅲ期1例，T3aN1M0/Ⅲ期1例，T4N1M0/Ⅳ期2例。前期术后有4例RCC患儿接受α-干扰素治疗，后均因不良反应而终止治疗。无化疗病例。患儿随访0.4~8.7年，中位随访时间1.3年。1例失访，15例存活，5例死亡，接受NSS的5例患者均存活。结论 儿童RCC较少见，因无特异性临床表现和影像学特征，术前病理诊断较困难，术后需要进行TFE3基因检测以明确病理类型。治疗以根治性肾切除术为主，对于肿瘤局限的合适病例亦可选择保留肾单位肾肿瘤切除术，以尽可能保留肾功能。

Renal cell carcinoma (RCC) in children: clinical characteristics and surgical treatment

Objective To investigate the characteristics,clinical manifestations, pathological features, surgical treatment and curative effect of renal cell carcinoma (RCC) in children. Methods A retrospective review and clinical analysis of 21 patients of RCC treated in Children's Hospital from Aug.2008 to Aug.2018 was undertaken in this study.The median age was 6.1 (range 1.2 to 10.8) years old.Fourteen boys and 7 girls.There were 13 cases of left kidney and 8 cases of right kidney.Among them,6 cases had painless hematuria,3 cases had abdominal pain,and 2 cases had abdominal masses as the first symptom,and the other 10 cases were found by color Doppler ultrasound in physical examination. Results Surgical treatments were performed in all 21 patients.Radical nephrectomy was performed in 14 cases,and retroperitoneal lymph node dissection was performed in 6 cases at the same time. Nephron-sparing surgery (NSS) was performed in 5 cases,and the maximum diameter of tumor was less than 4 cm in 4 cases and 7.8 cm in 1 case.Tumor biopsy was performed in 2 cases.According to pathological feature,9 cases were RCC,1 case was cystic RCC,and Xp11.2 translocation RCC were identified in 11 patients.According to TNM stage,10 cases were stage Ⅰ,4 stage Ⅱ,5 stage Ⅲ and 2 stage Ⅳ.Four cases received postoperative interferon,but were terminated owing to undesirable side effects.There was no chemotherapy cases.The median follow-up time was 1.3 (range 0.4 to 8.7) years.One case was withdrawn and there were 15 survivors and 5 cases of postoperative deathly.All 5 patients who received NSS survived. Conclusions RCC is rare in children.Because of the lack of specific clinical and imaging features,it is difficult to diagnose RCC before operation.TFE3 gene detection is needed to determine the pathological type after operation. Nephrectomy is the major treatment for RCC and nephron sparing surgery could be a reasonable option for patients with early localized RCC to protect the renal function.