Bilateral Wilms tumors: Treatment results from a single center

Özet

The management of bilateral Wilms tumor (BWT) is challenging, particularlydue to its presentation at a younger age, rarity, and difficulty for treatmentdecisions and surgical evaluation comparing to unilateral WT. In this study,the outcome of BWT patients from a single center who were treated bythe Turkish Pediatric Oncology Group (TPOG) Wilms Tumor Regimen wereretrospectively reviewed. From 1990 to 2016, 30 patients with synchronousBWT were treated with a preoperative chemotherapy of vincristine andactinomycin-D (VA). Chemotherapy was continued until safe nephronsparing surgery (NSS) could be performed for as long as radiological tumorresponse continued; otherwise, the chemotherapy was intensified by addingdoxorubicin (D) alternating with VA every 6 weeks. The median followup of patients was 59 months (4-297 months). The median duration ofpreoperative chemotherapy was 81 days and ranged between 14 days and198 days. Preoperative chemotherapy was modified in seven patients (23%)to the VAD regimen. Twenty-two patients (73%) had a radical nephrectomyon the larger tumor and NSS on the contralateral kidney, and 6 patients(20%) had bilateral NSS. Postoperative tumor stages for stage I, II and IIIwere 60%, 22% and 14%, respectively. The 5-year event free survival (EFS)rates were 100%, 90% and 51% for stages I, II and III (p=0.02), respectively.Unfavorable histology and nephrogenic rests were reported in 20% and 20%of patients, respectively. The 5-year overall survival (OS) and EFS rates were50% and 25%, respectively, in patients with anaplasia, while the same rateswere 96% and 96% in patients with favorable histology tumors (p=0.05 andp<0.001). The 10-year EFS and OS rates for all patients were 82% and 86%,respectively. Our results are comparable with the literature. VA is effectiveas initial preoperative treatment of BWT and allows for safe resection.