成人肾母细胞瘤的治疗及其长期随访结果

Long-term follow-up and therapy of adult Wilms' tumor

目的:分析并报道成人肾母细胞瘤的临床特征、治疗及长期随访结果。方法:对1970年1月至2011年12月就诊于北京大学第一医院泌尿外科的成人肾母细胞瘤患者进行回顾性研究,所有患者均经病理证实,对临床表现、肿瘤分期、手术情况、病理结果、术后辅助治疗情况及预后进行分析。所有患者均进行定期随访,并统计随访时间、肿瘤特异性生存时间及肿瘤特异性生存率。结果:通过对病例资料进行回顾,共有10例患者纳入本研究。患者中位年龄33.5岁,男、女各5例,左侧4例,右侧6例,其中80%的患者因临床症状而就诊,30%的患者以腹部肿物为首发症状,30%的患者在就诊时即已经存在远处转移。肿瘤分期为Ⅰ、Ⅱ、Ⅲ和Ⅳ期的患者分别为2、1、4和3例。除1例患者只接受肾肿瘤穿刺活检外,其余患者均接受手术治疗。随访时间12～187个月,中位随访时间20个月,其中1例患者失访,5例患者已经死亡,2例患者出现肿瘤复发并带瘤生存,2例患者无复发生存,中位生存时间42个月。结论:长期随访数据显示,成人肾母细胞瘤的预后较差,较早的肿瘤分期及手术结合放、化疗的综合治疗是患者改善预后的关键因素。

Objective：To investigate and analyze the treatment and long-term follow-up results of adult Wilms＇ tumor. Methods： Medical records for diagnosed Wilms＇ tumor in patients aged more than 15 years from Jan. 1970 to Dec. 2011 were reviewed retrospectively. The clinical presentations, stages, operative details, pathologic findings, adjuvant therapies and outcomes were analyzed. All the patients received regular follow-up, and particular attention was paid to the median follow-up period and tumor specific survival time. Results： The records revealed the 10 patients with a median age of 33.5 year. Male and female were each 5. Left was 4 and right was 6. In the study, 80% of the patients had clinical presentations, and 30% of the patients presented with abdominal mass, and 30% of the patients had distant metastasis at the time of diagnosis. The number of the patients with tumor stages I , II , III, and IV were 2, 1,4, and 3. One patient just underwent fine needle biopsy due to advanced tumor stage, and the others underwent surgical operations. The range of the follow-up time was 12 to 187 months, and the median follow-up period was 20 months. One patient lost the follow up , 5 patients died, 2 patients sur- vived with tumor recurrence, and 2 patients survived without tumor recurrence. The median survival period was 42 months, and one patient lost the follow-up. Conclusion： The long-term follow-up data demonstrated the poor prognosis of adult Wilms＇ tumor. Early tumor stage and the combination of operation, radiotherapy and chemotherapy are key factors to improve the outcomes.