Bilateral Wilms tumors (WT) treated with the SIOP 93 protocol in France: Epidemiological survey and patient outcome

Background The treatment of bilateral Wilms tumors (WT) requires multimodality therapy with individualized decision to ensure cure while preserving as much renal parenchyma as possible. Procedure We analyzed the clinical records of 49 children with bilateral WT treated in France between 1993 and 2001, according to the SIOP-93 guidelines (individual treatment program: Treatment was continued as long as there was imaging evidence of tumor regression). Pathology reports, duration of preoperative chemotherapy and surgical records were also reviewed. Overall Survival (OS) and Event-Free Survival (EFS) rates were studied and relationships between possible prognostic factors and survival were assessed. Results Imaging studies revealed bilateral involvement in 98% of the cases. Whatever the response to preoperative chemotherapy, the mean duration of neoadjuvant chemotherapy was 80 days (Q1Q3: 4789 days). Forty-eight children underwent nephron sparing surgery (NSS) at least for one kidney and 19 for both. Five-year EFS and OS rates were, respectively, 83.4 and 89.5%. Only the most advanced stages were shown to affect OS (P?=?0.03). At study endpoint, end-stage renal disease (ESRD) was reported in seven children, associated with a predisposing phenotype in three. Conclusions Results of this study demonstrate a favorable outcome of patients with bilateral WT receiving an individual treatment program. With a tailored approach to treatment according to the tumor response, 77% of our patients were operated before the third month of preoperative chemotherapy. In spite of good survival, 14% of our patients have ESRD. Pediatr Blood Cancer 2012; 59: 5761. (C) 2012 Wiley Periodicals, Inc.