Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children's Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist's assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively.Survival data were analyzed by Kaplan-Meier.Log-Rank analysis was used for univariate analysis. Result: One case was excluded because of giving up the therapy even though no disease progress was identified. A total of 15 cases (5 males and 10 females) were included in this study. The median age at diagnosis was 3.8 years (range 0.5-9.1 years). The tumor staging at diagnosis included one case of stageⅠ, 7 cases of stageⅡand 7 cases of stage Ⅲ. Among cases of stage Ⅲ, 6 cases had radiation therapy history. The pathology of all patients' recurrent tumor was favorable histology (FH). The median follow-up time was 34.6 months (range 12.5-132.7 months) until March 21, 2017. The time from initial diagnosis to relapse was 7.9 months (range 3.1-17.9 months). Four cases experienced local recurrence, 9 cases relapsed with metastases (6 cases in lungs, 2 in livers, 1 in mediastinum) and 2 cases relapsed in both local site and with metastases. Except to 2 cases received irregular retreatment, 13 cases received regimen I (doxorubicin, vincristine, epoposide and cyclophosphamide for 25 weeks) as relapsed chemotherapy. Five cases received autologous bone marrow transplantation (ABMT). Until the last follow-up, 8 cases achieved continuous complete remission (range 6.7-104.3 months), 3 cases had relapse again or progressing and 4 cases died. The estimated 5-year overall survival (OS) rate and event free survival (EFS) rate were (70±15)% and (52±15)%. According to whether received ABMT or not, the 5-year EFS rate were 51% and 53%. According to whether relapsed within 6 months after diagnosis or not, the 5-year EFS rate were 38% and 56% respectively. Conclusion: The 5-year EFS rate of pediatric relapsed FH WT have reached above 50% by multi-disciplinary treatment in our experience and we encourage patients and doctors to receive retreatment.
目的： 总结儿童肾母细胞瘤(WT)复发后再治疗的远期疗效。 方法： 收集2006年4月到2016年6月上海儿童医学中心收治的16例儿童复发性WT病例资料，均通过病理、影像学、肿瘤内外科协助诊断，采用外科手术、内科化疗、选择性放疗综合治疗措施。回顾性研究其临床特征、远期预后和预后相关因素。生存资料分析采用Kaplan-Meier生存分析法。单因素分析采用Log-Rank检验。 结果： 剔除复发后治疗过程中未进展，但家长放弃继续治疗1例，共15例复发患儿列入统计。男5例，女10例，年龄3.8(0.5～9.1)岁。首次诊断时分期为Ⅰ期1例、Ⅱ期7例、Ⅲ期7例。Ⅲ期7例中6例有放疗史。复发病理类型均为预后良好型(FH)。截至2017年3月21日，随访时间34.6(12.5～132.7)个月。初诊到复发经历时间7.9(3.1～17.9)个月。4例原发部位复发，9例转移性复发(肺6例、肝2例、纵隔1例)，2例同时有原位及其他部位转移复发。复发后2例接受不规则化疗，余13例均按复发后方案(I方案：阿霉素、长春新碱、依托泊苷、环磷酰胺治疗25周)化疗，5例行自体造血干细胞移植(ABMT)。截至末次随访，8例仍在持续完全缓解中[(6.7～104.3)个月]，3例再次复发或进展，4例死亡。5年总生存率(OS)为(70±15)%，5年无事件生存率(EFS)为(52±15)%。患儿ABMT与否的5年EFS分别为51%和53%。患儿是否初诊6个月内复发的5年EFS分别为38%和56%。 结论： 儿童复发性WT FH型采用手术及放化疗综合治疗方式再治仍可有50%以上的远期EFS，应鼓励患者及医务人员再治疗。.
Keywords: Child; Hematopoietic stem cell transplantation; Recurrence; Wilms tumor.