Bilateral Wilms' tumors: single-center experience with 22 cases and literature review

Abstract

Objectives: Bilateral Wilms' tumors represent a therapeutic challenge. The primary aim of management is eradication of the neoplasm and preservation of renal function. We present our experience in the management of such cases in a single-center experience.

Methods: This was a retrospective study of 22 patients with histologically proven bilateral nephroblastoma who were treated from 1993 to 2008 at our center. Of the 22 patients, 12 were girls and 10 were boys, with a median age of 3 years (range 1-9); 19 had a synchronous presentation and 3 a metachronous presentation. Of the 22 patients, 6 underwent initial surgical resection followed by chemotherapy and 16 underwent initial biopsy and preoperative chemotherapy. The final oncologic and renal outcomes were assessed.

Results: The median follow-up period was 3 years (range 1-11). Of the 22 patients, 8 died, for an overall survival rate of 63.5%. The survival for the initial chemotherapy and initial surgery groups was essentially similar. Of all the variables studied, unfavorable histologic findings had a significant negative effect on survival. Of the 5 patients with unfavorable histologic findings, 4 died during the follow-up period. The median volume of preserved renal parenchyma was 40%. All patients had good renal function during follow-up, except for 1 patient who had undergone bilateral nephrectomy.

Conclusions: Bilateral Wilms' tumors impose 2 conflicting issues: elimination of the pathology and preservation of the renal function. Currently, treatment regimens involving initial chemotherapy followed by conservative surgery can achieve these goals in an important proportion of patients.