成人肾母细胞瘤的诊疗及预后分析(附10例报告)

Diagnosis,treatment and prognosis of adult wilms' tumor

目的:探讨成人肾母细胞瘤的诊疗方法和预后。方法:回顾性分析10例成人肾母细胞瘤患者的临床资料。男8例,女2例,年龄16～62岁,平均29岁。就诊症状为单纯肉眼血尿3例、腰腹部肿块或腰腹痛4例(伴血尿2例)、无症状查体发现3例。患者均行B超、IVU、尿脱落细胞学检查,8例行CT平扫+强化检查。影像学检查提示肾占位6例、肾盂占位3例、输尿管占位1例。CT提示肾门或腹腔淋巴结肿大4例。10例均行手术治疗,根治性肾切除6例,患肾、输尿管全段切除+膀胱输尿管口袖口状切除4例,2例根治性肾切除患者因肿物与周围粘连严重切除部分腹膜。行腹膜后淋巴结清扫5例。结果:所有病例均于术后病理确诊为肾母细胞瘤,预后良好组织型(FH)4例,预后不良组织型(UH)6例,病理证实淋巴结转移3例。按美国国家肾母细胞瘤研究组(NWTS)分期标准,分别为Ⅰ期4例、Ⅱ期3例、Ⅲ期3例。7例患者接受术后辅助放化疗。随访9例患者1～13年,5例无瘤生存,生存时间为1.5～13年,平均4.4年。1例术后1年死于肿瘤肺转移,1例术后1年死于肿瘤脑转移,2例术后2年内局部复发,再次接受手术。结论:成人肾母细胞瘤为罕见的恶性肿瘤,预后较差,确诊主要依靠病理诊断,目前尚无公认的最佳治疗手段。手术治疗并辅以合理、及时的放疗、化疗是改善其预后的有效措施。

Objective：To study the diagnosis and treatment of adult Wilms＇tumor and to improve the survival rate of adult Wilms＇tumor. Method： A retrospective study was conducted on 10 patients who were diagnosised as wilms＇tumor in our hospital. Of them, there were 8 male patients and 2 female patients. They were 16 to 62 years old, and their mean age was 29 years old. Three patients had gross hematuria as their singular clinical manifesta tions. Four patients got abdominal and flank pain or obvious lumps（two of them had gross hematuria）. Three cases were found in routine check. Five cases were diagnosised as renal spac-occupying lesions preoperation. CT showed lymph node swelling infour cases. Four cases were dJagnosised as space-occupying lesion in renal pelvis preopera tion. There was one ease was diagnosised as ureteral space-occupying lesion preoperation. Six patients successfully underwent nephreetomy. Four patients accepted nephroureterectomy. Because of tumor adhesion, we cut part of peritoneal in two cases. Retroperitoneal lymph node elimination were performed in five patients. Result： All pa tients had pathological diagnosis of Wilms＇tumor. Four cases were classified into favorable histologic type, and six in unfavorable histologic type. Three patients with lymph node metastasis. According to the national Wilms＇tumor study staging system, 4 cases in stage I , 3 cases in stage II , 3 cases in stage III. Three cases accepted chemo- therapy plus radiotherapy after operation. Four patients accepted chemotherapy only. Five patients survived with no tumor from 1.5 to 13 years, in average of 4.4 years. One case died from tumor pulmonary metastasis in 1 year after operation. One case died from brain metastasis in 1 year after operation. Two patients were found tumor recru- desced after the operation for two years and all accepted operation again. Conclusion： Wilms＇tumor is rare in adults. The prognosis of Wilms＇tumor in adults is worse than that in children. The diagnosis of adult Wilms＇tumor depends on pathology. Advisable and timely treatments such as chemotherapy and radiotherapy are crucial for the prognosis of Wilms＇tumor in adults.