中文摘要：

目的 总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗和预后,提高对该病的认识.方法 回顾性分析2011年1月至2015年10月我院收治的5例输尿管肿瘤患儿的临床资料,结合文献总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗及预后.5例患儿均为男性,输尿管炎性肌纤维母细胞瘤3例,2例以腹痛就诊,分别为6岁和10岁,肿瘤位于输尿管下段,均行输尿管下段肿物切除＋输尿管端端（输尿管膀胱）吻合,1例以尿痛并血尿就诊,年龄1岁9月龄,肿物来源于下段输尿管,经输尿管膀胱连接部长入膀胱,继发膀胱输尿管积水,经输尿管和膀胱联合切除肿物＋输尿管膀胱再植术.结果 术后随访5~48个月,未见肿瘤复发及转移.恶性横纹肌样瘤1例6岁,以左下腹痛2周就诊,输尿管肿瘤边界不清与腰大肌肉和后腹膜粘连,切除肿物行输尿管端端吻合,术后ICE方案化疗一疗程后局部复发,放弃治疗,术后233d死亡.尤文/原始神经外胚层瘤1例12岁,以右侧腰痛10d就诊,肿瘤位于输尿管髂血管水平,行输尿管肿瘤切除术＋输尿管端端吻合术,术后予CAV＋IE化疗12个月,随访56个月,未见转移及复发.结论 输尿管肿瘤在儿童发病率极低,文献曾报道的病理类型包括炎性肌纤维母细胞瘤、恶性横纹肌样瘤、尤文/原始神经外胚层瘤、横纹肌肉瘤,术前无特异方法诊断,手术完整切除肿瘤并重建输尿管是治疗的主要方法,确诊需要结合病理检查,根据病理类型决定是否化疗,治疗后均需要长期随访.

英文摘要：

Objective To explore the clinical manifestations,diagnosis,treatment and prognosis of pediatric ureteral tumors.Methods Retrospective analyses were conducted for clinical data of 5 cases of ureteral tumors from January 2011 to December 2015.And the relevant literature was reviewed.Results All five patients were males.Three cases were confirmed pathologically as inflammatory myofibroblastic tumor.Two of them,aged 6 and 10 years respectively,were admitted for abdominal pain.Tumor was located at distal ureter and complete tumor resection and ureteral anastomosis were performed.Another patient manifested dysuria and hematuresis.Tumor also originated in distal ureter and metastasized into urinary bladder through ureterovesical junction.Removal of tumor and ureteric reimplantation were performed.During a follow-up period of 5-48 months,there was neither recurrence nor metastasis.A 6-year-old boy of malignant rhabdoid tumor presented with left abdominal pain.Tumor had an obscure margin with psoas and posterior peritoneum.Resection and ureteroureterostomy were performed after one chemotherapeutic course of ICE.However,neoplasm recurred 1 month later and he died at 233 days after operation.A 12-year-old boy of Ewing＇s sarcoma or primitive neuroectodermal tumor presented with right loin pain.Tumor was located at distal ureter and complete tumor resection and ureteroureterostomy were performed after 12-month alternate CAV＋IE chemotherapy.During a follow-up period of 56 months,there was no onset of recurrence or metastasis.Conclusions Pediatric ureteral tumor is a rare disease.And its diverse pathological forms include inflammatory myofibroblastic tumor,malignant rhabdoid tumor,Ewing＇s sarcomas/primitive neuroectodermal tumor and rhabdomyosarcoma.No specific method is available for a definite preoperative diagnosis.Selecting chemotherapy and radiotherapy depends upon the type of pathology.And long-term follow-ups should be employed after operation.