摘要目的:探讨儿童马蹄肾合并肾母细胞瘤合理的诊治方法。方法:回顾性分析2007～2018年我院收治7例马蹄肾合并肾母细胞瘤患儿的临床资料。其中男3例，女4例，就诊年龄1岁至8岁8个月，平均4岁6个月。肿瘤位于左侧4例，右侧2例，峡部1例。主要临床表现：腹痛4例，无症状腹部包块1例，偶然超声检查发现肿物1例，外院手术并放化疗后肿瘤复发1例。4例(其中2例因瘤体破裂，1例因瘤体巨大且合并肺部转移，1例因瘤体巨大)术前化疗后行患侧肾脏切除及峡部肿瘤切除术，术后辅以放疗＋化疗；2例肿瘤位于肾脏一极行肿瘤剜除术，术后辅以化疗；外院术后及放化疗后肿瘤复发1例于我院行复发肿瘤切除术＋盆腔转移灶切除。结果:5例获随访(含2例肾肿瘤剜除术)6个月至11年，平均44个月，均未发现肿瘤复发，行肿瘤剜除者肾脏功能良好。2例失访，包括外院术后及放化疗后肿瘤复发1例，术前肺转移1例。结论:马蹄肾合并肾母细胞瘤较少见，根据肿瘤位置及大小选择个体化治疗方式，如肿瘤位于肾脏一极可行保留肾脏的肿瘤剜除术，术后按病理分型分期辅以化疗和/或放疗。

abstractsObjective:To explore the diagnosis and treatment of horseshoe kidney with pediatric Wilms tumor.Methods:The clinical data were analyzed retrospectively for 7 children with horseshoe kidney complicated with Wilms tumor. There were 3 boys and 4 girls with an average age of 54(12-104) months. The locations of tumors were left (n=4), right (n=2) and isthmus (n=1). The major clinical manifestations included abdominal pain (n=4), asymptomatic abdominal mass (n=1), incidental ultrasonically detected mass (n=1) and postoperative tumor recurrence after chemoradiotherapy at another hospital (n=1). Two cases of tumor rupture, 1 case of huge tumor with pulmonary metastasis and 1 case of huge tumor received preoperative chemotherapy and partial nephrectomy followed by chemoradiotherapy. Two cases of tumor in one pole of kidney underwent nephron-sparing surgery followed by chemotherapy. One case of postoperative tumor recurrence and chemoradiotherapy underwent unilateral nephrectomy, isthmic tumor resection and lymph node dissection.Results:Five children (including 2 cases of nephron-sparing surgery) were followed up for an average of 44(61-132) months. Two cases were lost to follow-ups, including tumor recurrence after surgery and chemotherapy (n=1) and preoperative lung metastasis (n=1).Conclusions:Horseshoe kidney with pediatric Wilms tumor is so rare that individualized treatment should be based upon its location and size. And pathological typing guides supplemental chemoradiotherapy.