儿童非肾母细胞瘤肾脏肿瘤的临床治疗探讨

目的总结和探讨儿童非。肾母细胞瘤肾脏肿瘤的治疗方法。方法1998年7月至2010年7月我们共收治非肾母细胞瘤肾脏肿瘤患儿18例，其中男性14例，女性4例，年龄最大9岁，最小2个月，平均年龄（40．17±34．04）个月，术后病理诊断包括肾透明细胞肉瘤9例（9／18，50％），肾横纹肌样瘤2例（2／18，11．1％），肾癌2例（2／18，10．1％），肾中胚层。肾瘤1例（1／18，5．56％），后。肾间质瘤1例（1／18，5．56％），后肾腺瘤1例（1／18，5．56％），肾血管平滑肌脂肪瘤1例（1／18，5．56％），肾原始神经外胚叶瘤1例（1／18，5．56％）。结果随访9例肾透明细胞肉瘤，8（8／9）例无瘤生存，最长随访时间5年，最短2个月，平均随访时间（24．56±17．27）个月，1例手术化疗后下腔静脉瘤栓残留水平至右心房入口，放弃治疗1．5年后死亡。2例肾横纹肌样瘤经手术、放疗及化疗，随访3年和4年均无瘤存活。2例。肾癌单纯手术切除，密切随访25个月、13个月无复发及转移。1例。肾原始神经外胚叶瘤经手术及化疗，随访16个月无瘤存活。1例肾中胚层肾瘤、1例后。肾间质瘤、1例后。肾腺瘤、1例肾血管平滑肌脂肪瘤均完整切除，现最长随访时间4年，最短1年，无一例复发。结论儿童非肾母细胞瘤肾脏肿瘤所占比例虽小，但临床鉴别诊断困难，病理诊断非常重要，手术、化疗及放疗综合治疗是诊治的首要选择。

The management of non-wilms renal tumors in children.

Objetive To present our experience of managing non-wilms, renal tumors in children. Methods During From July 1998 to July 2010, 18 eases of non-wilms, renal tumors in children were treated. 14 cases were male, 4 cases were female, age ranged from 9 years to 2 months, mean age was 40.17 ± 34.04 months. The pathological diagnosis included 9/18（50% ） cases of clear cell sarcoma ,2/18（ 11.1% ）cases of rhabdoid tumor of kidney, 2/18 （ 10. 1% ） cases of renal cell carcinoma, 1/18 （ 5.56% ） case of mesoblastic nephroma., 1/18（5.56% ）case of metanel？hric stromal tumor,I/18（5.56% ）case of metanephric adenoma, 1/ 18（5.56% ）cases of angiomyolipoma, 1/（!8 （5.56%）ease of primitive neuroectodermal tumor. Results 8/ 9cases of clear cell sarcoma cases were survival, whose average follow-up time was 24.56 ± 17.27 months, 1 case died after giving up treatment 1.5 year. 2/cases of rhabdoid tumor of kidney were survived after surgery and chemotherapy, follow-up time was 3 years and 4 years. 2 cases of renal cell carcinoma were survived after surgery, follow-up time was 13 months and 25 months. 1 case of primitive neuroectodermal tumor was survived after surgery and chemotherapy, follow-up time was 16 months. 1 case of mesoblastic nephroma, metanephrlc stromal tumor , metanephrie adenoma and angiomyolipoma survived without relapse. Conclusions Although non-wilms renal tumors is a small part of all kidney tumor, it is difficult to identify from other tumors. So patho- logical diagnosis is essential. Surgical treatment combined with radiotherapy and chemotherapy is essential.