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

Pediatr Blood Cancer. 2012 Jul 15;59(1):57-61. doi: 10.1002/pbc.24059. Epub 2012 Jan 11.


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 (Q1-Q3: 47-89 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.

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • France / epidemiology
  • Humans
  • Kidney Neoplasms / mortality*
  • Kidney Neoplasms / therapy*
  • Male
  • Preoperative Care*
  • Retrospective Studies
  • Survival Rate
  • Wilms Tumor / mortality*
  • Wilms Tumor / therapy*