儿童马蹄肾合并肾母细胞瘤的诊治分析

Diagnosis and treatment of horseshoe kidney with pediatric Wilms tumor

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。

Objective 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.