儿童肾细胞癌的临床、病理特点及预后分析

Renal cell carcinoma in children： a clinicopathologic study

目的 总结小儿肾细胞癌的临床病理特点和远期疗效. 方法 回顾性分析1973年1月至2012年3月收治的29例儿童肾细胞癌患者的临床资料.患儿年龄2.5～ 16.0岁,平均9.6岁.男16例,女13例.左侧16例,右侧13例.无痛肉眼血尿17例（3例为外伤后血尿）,血尿＋腹部包块3例,腹部包块3例,腹痛2例,血尿＋腹痛1例,B超检查偶然发现3例. 结果 29例中3例因肿瘤直径＜7 cm行保留肾单位手术；1例因肿瘤直径15 cm包绕腹主动脉和下腔静脉行肿瘤姑息切除；1例肿瘤巨大,最大径25 cm,术中有肉眼残留；余24例均行根治性肾切除术.肿瘤直径2.5～25.0 cm,平均6.8 cm.T1N0M0 16例,T1N1M0 5例,T2N1M0 3例,T3N1M0 2例,T4N1M0 2例,T4N1M11例.病理检查示Xp11.2易位相关肾癌21例,其中淋巴结转移11例（52.4％）,透明细胞癌6例,乳头状癌2例.21例获随访,其中Xp11.2易位相关肾癌13例、透明细胞癌6例、乳头状癌2例.随访时间1.5 ～ 34.0年,平均12.3年.3例（均为Xp11.2相关肾癌,T1N0M0 1例,T4N1M12例）肿瘤复发后死亡,18例（T1N0M011例,T1N1M02例,T2N1M03例,T3N1M01例和T4N1M11例）无瘤存活. 结论 儿童肾细胞癌少见,多见于5岁以上儿童,血尿为主要症状.Xp11.2易位相关肾癌为主要病理类型,易出现局部淋巴结转移,分期高,但在儿童表现为生物活性惰性.手术切除是主要的治疗方法,对于肿瘤直径＜7 cm者主张行保留肾单位手术.对于Ⅰ、Ⅱ期和T1-2N1M0的局限性肾细胞癌可仅行手术切除,术后无需辅助治疗.

Objective To discuss the unique biological, histological and clinical features of pediat- ric renal cell carcinoma （ RCC）. Methods A retrospective review and biological analysis of all RCC cases presenting to our hospital from January 1973 to March 2012 was undertaken. Results Twenty-nine RCC pediatric patients （16 boys, 13 girls） with mean age of 9.6 （range 2.5-16.0） years were identified. The presentations included hematuria in 17 （58.6%） cases with 3 who developed hematuria after trauma, ab- dominal mass with hematuria in 3 （10.3%） , abdominal mass in 3, abdominal pain in 2, abdominal pain with hematuria in 1, and incidentally finding in 3. The diameter of tumor was from 2.5 cm to 25.0 cm, mean 6.8 cm. According to TNM stage grouping system, 16 cases were stage I, 10 stage III, and 3 stage IV. Xpl 1.2 translocation RCC was identified in 21 patients, clear cell RCC 6, papillary RCC 2. Of the 29 cases, 3 patients with the tumor less than 7-cm had nephron-sparing surgery. A 15-cm tumor was incompletely re- moved in 1 patient and another patient with a 25 crux 18 cmx 15 cm tumor had gross residual. Nephrectomy was performed for the affected kidney in the remaining 24 patients. Twenty-one patients （Xpll.2 transloca- tion RCC 13 patients, clear cell RCC 6, papillary RCC only 2） were followed up from 1.5 to 34.0 years, 18 were living well （T1NoMo in 11 cases, T1NIMo in 2, T2NIMo in 3, T3N1Mo in 1 and T4N1Ml in 1） and 3 died of recurrence. Conclusions Ahhough RCC is rare in children, pediatric RCC behaves in a distinct fashion compared with adult forms of RCC. Hematuria is the main symptom in pediatric RCC. Xpl 1.2 trans- location RCC is the predominant form, associated with an advanced stage at diagnosis. Nephrcctomy is the common treatment for RCC and nephron sparing surgery could be a reasonable option for patients with tumor smaller than 7 cm. For localized RCC （T1-2N0-1 M0） , simple kidney removal surgery is sufficient for treat- ment without lymph node dissection and postoperative adjuvant treatment.