Bilateral Wilms tumors: Treatment results from a single center.

Abstract

Aydın B, Akyüz C, Yalçın B, Ekinci S, Oğuz B, Akçören Z, Yıldız F, Varan A, Kurucu N, Büyükpamukçu M, Kutluk T. Bilateral Wilms tumors: Treatment results from a single center. Turk J Pediatr 2019; 61: 44-51. The management of bilateral Wilms tumor (BWT) is challenging, particularly due to its presentation at a younger age, rarity, and difficulty for treatment decisions and surgical evaluation comparing to unilateral WT. In this study, the outcome of BWT patients from a single center who were treated by the Turkish Pediatric Oncology Group (TPOG) Wilms Tumor Regimen were retrospectively reviewed. From 1990 to 2016, 30 patients with synchronous BWT were treated with a preoperative chemotherapy of vincristine and actinomycin-D (VA). Chemotherapy was continued until safe nephron sparing surgery (NSS) could be performed for as long as radiological tumor response continued; otherwise, the chemotherapy was intensified by adding doxorubicin (D) alternating with VA every 6 weeks. The median followup of patients was 59 months (4-297 months). The median duration of preoperative chemotherapy was 81 days and ranged between 14 days and 198 days. Preoperative chemotherapy was modified in seven patients (23%) to the VAD regimen. Twenty-two patients (73%) had a radical nephrectomy on the larger tumor and NSS on the contralateral kidney, and 6 patients (20%) had bilateral NSS. Postoperative tumor stages for stage I, II and III were 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 were 50% and 25%, respectively, in patients with anaplasia, while the same rates were 96% and 96% in patients with favorable histology tumors (p=0.05 and p < 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 effective as initial preoperative treatment of BWT and allows for safe resection.

• Authors: Burça Aydın, Canan Akyüz, Bilgehan Yalçın, Saniye Ekinci, Berna Oğuz, Zuhal Akçören, Ferah Yıldız, Ali Varan, Nilgün Kurucu, Münevver Büyükpamukçu, Tezer Kutluk
• Journal: The Turkish journal of pediatrics (Turk J Pediatr) Vol. 61 Issue 1 Pg. 44-51 ( 2019) ISSN: 2791-6421 [Electronic] Turkey
• PMID: 31559721 (Publication Type: Journal Article)

Chemical References

• Antineoplastic Agents
• Dactinomycin
• Vincristine
• Doxorubicin

Topics

• Antineoplastic Agents (therapeutic use)
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Chemotherapy, Adjuvant
• Child
• Child, Preschool
• Dactinomycin (therapeutic use)
• Doxorubicin (therapeutic use)
• Female
• Follow-Up Studies
• Humans
• Infant
• Kidney Neoplasms (mortality, pathology, therapy)
• Male
• Neoadjuvant Therapy
• Neoplasm Staging
• Nephrectomy (methods)
• Retrospective Studies
• Survival Analysis
• Treatment Outcome
• Turkey
• Vincristine (therapeutic use)
• Wilms Tumor (mortality, pathology, therapy)