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目的:探讨保留肾单位手术(nephron-sparing surgery,NSS)在儿童单侧肾脏肿瘤中的应用情况。方法:回顾性分析2007年1月~2015年7月于我院接受NSS的26例单侧肾脏肿瘤患儿的临床资料(其中男13例,女13例,发病年龄0.5~17.1岁,中位年龄4.8岁)并进行随访。结果:随访成功25例,失访1例。均通过腹部超声及腹部增强CT做出临床诊断。肿瘤位于肾脏一极者19例(上极8例,下极11例),位于肾脏中央者5例,马蹄肾1例(肿瘤位于左肾下极与岬部之间),位于肾盂内1例。均接受经腹入路开放性NSS。术后病理:肾母细胞瘤4例(其中马蹄肾合并肾母细胞瘤1例,右侧孤立肾合并肾母细胞瘤1例),恶性横纹肌样瘤1例,肾细胞癌5例(其中Xp11.2易位/TEF3基因融合相关性肾癌3例),婴儿骨化性肾肿瘤1例,中胚叶肾瘤1例,后肾腺瘤1例,多囊性肾发育不良2例,多囊性肾瘤5例,多房性肾囊肿1例,单纯性肾囊肿1例,肾素瘤1例,炎性肌纤维母细胞瘤1例,嗜酸细胞腺瘤1例,炎症1例。4例肾母细胞瘤及1例恶性横纹肌样瘤术后均按Ⅲ期给予化疗+放疗,其余患儿术后均未接受化、放疗。随访时间14~108个月。25例获访病例均无瘤存活。结论:在单侧肾脏肿瘤患儿中可以选择性地使用NSS,特别是有潜在肾功能异常以及良性肿瘤患儿。对于单侧肾脏恶性肿瘤患儿,是否可行NSS,目前仍有争议。
Objective: To investigate the application of nephron-sparing surgery (NSS) in unilateral renal tumors in children. Methods: The clinical data of 26 children with unilateral renal tumors who received NSS in our hospital from January 2007 to July 2015 were retrospectively reviewed (13 males and 13 females, with a mean age of onset of 0.5 to 17.1 years Age 4.8 years) and followed up. Results: Follow-up was successful in 25 cases and lost in 1 case. All through abdominal ultrasound and abdominal enhanced CT to make a clinical diagnosis. Tumor in the kidneys of a pole in 19 cases (upper pole in 8 cases, lower pole in 11 cases), located in the middle of the kidney in 5 cases, horseshoe kidney in 1 case (tumor located in the left kidney between the lower pole and the promontory), located in the pelvis in 1 case . All patients underwent transabdominal open NSS. Postoperative pathology: Wilms tumor in 4 cases (including horseshide kidney with nephroblastoma in 1 case, isolated right kidney with nephroblastoma in 1 case), malignant rhabdomyosarcoma in 1 case, renal cell carcinoma in 5 cases (including Xp11 .2 translocation / TEF3 gene fusion-related renal cell carcinoma in 3 cases), 1 case of ossified kidney neoplasms in infancy, 1 case of mesodermal nephroma, 1 case of adenocarcinoma of the kidney, 2 cases of polycystic kidney dysplasia 5 cases of cystic nephroma, 1 case of multilocular renal cyst, 1 case of simple renal cyst, 1 case of renin, 1 case of inflammatory myofibroblastoma, 1 case of eosinophilic adenoma and 1 case of inflammation. 4 cases of nephroblastoma and 1 case of malignant rhabdomyosarcoma were treated with chemotherapy and radiotherapy according to the third stage, and the rest of the cases were not accepted and radiotherapy after operation. Follow-up time was 14 to 108 months. Twenty-five cases were tumor-free survival. CONCLUSIONS: NSS can be selectively used in children with unilateral renal tumors, especially in children with underlying renal dysfunction and benign tumors. For unilateral renal cancer in children, is feasible NSS, is still controversial.