Impact of surgery and chemotherapy over renal function in bilateral Wilms tumor patients

Abstract

INTRODUCTION & OBJECTIVES: Bilateral Wilms’ tumor represents 3% of all cases. The challenge of this situation is curing these patients preserving their renal function and quality of life (dialysis free status). Our purpose was to evaluate the success of renal function preservation in children treated for bilateral Wilms’ tumor. MATERIAL & METHODS: From January/1982 through June/2009, 13 children with bilateral Wilms’ tumor (4F: 9M, median age 16 months) were diagnosed. All patients received preoperative chemotherapy with VCR/ACTD (13 patients), VCR/ACTD/ADRIA (10 patients), VCR/ACTD/ADRIA/CBDCA/ETO (3 patients), VCR/ACTD/ADRIA/CBDCA/ETO/IFO (2 patients), for 8 through 40 weeks (median 16 weeks). All patients were submitted to surgical procedure nephron sparing objective. RESULTS: Radical unilatera l nephrectomy was performed in 9 patients (combined with contralateral nodulectomy in 8), bilateral nodulectomy in 3, and unilateral nodulectomy in 1. Two patients presented unilateral disappearing of the disease after preoperative chemotherapy on image and surgical evaluation. The histhopatological analysis revealed the classic triphasic Wilms’ tumor in 9 patients, rhabdomyoblastic differentiation in 3 and blastematous rests in one patient. Four patients relapsed: 2 in the remaining kidney, 1 in the tumoral bed and 1 in lungs (9mo, 1mo, 5mo and 6 mo after surgery). A ll, except the lung relapsed patient, were rescued, achieving a second remission, but one presented a second diffuse abdominal relapse and died. 11/13 patients have normal renal function and are alive and free of disease for 23+ months to 28+years. 1/13 patients presents microalbuminuria and mild systemic hypertension, requiring medication support. CONCLUSIONS: Bilateral Wilms’ tumor was successfully treated and renal function was preserved in 84.6% of surviving patients, even those cases that received nephrotoxic drugs.