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

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

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1
Department of Pediatrics, Centre Oscar Lambret, 59020 Lille Cedex, France. h-sudour@o-lambret.fr

Abstract

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.

PMID:
22238153
DOI:
10.1002/pbc.24059
[Indexed for MEDLINE]
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