Surgical aspects, violations and outcomes of Wilms tumor-a multicenter study in a resource-limited country

摘要

Background:Wilms tumor is the commonest malignant renal neoplasm in children. Surgery plays a pivotal role in the management, and evidence-based guidelines for surgical resection have been established by the major international groups. Any deviation from the protocol is considered as a violation. The goal of this study was to evaluate outcomes of the patients with unilateral Wilms tumor treated at a developing country and to analyze surgical violations (SV) and their impact on the prognosis. A retrospective review was conducted for 37 patients who were presented to our hospitals and underwent nephrectomy for WT from January 2016 to December 2018. All participating centers adopt Children’s Oncology Group protocol. The SV were analyzed by logistic regression. Overall survival (OS) and event-free survival (EFS) were estimated by the Kaplan-Meier method.ResultsThere were 12 (32.4%), 11 (29.7%), 10 (27%), and 4 (10.8%) stages I, II, III, and IV, respectively. Their median age at time of diagnosis was 3.1?years. Upfront nephrectomy was performed for 30 cases. Six patients had tumor relapse (2 lungs and 4 local recurrences) at a median follow-up of 15.7?months. Out of the relapsed patients, two had unfavorable histology, and regarding their staging, four were stage III, one was stage II, and one was stage IV. Thirty-month OS and EFS were 84.3% and 81.1%, respectively. Twenty-seven SV occurred within 25 patients. Lack or inadequate lymph node sampling represented 74.07% (20/27), intraoperative tumor rupture and spillage accounted for 18.52% (5/27), and unwarranted preoperative biopsy happened in 7.41% (2/27). The SV were not correlated with mortality (p value = 0.381); however, they had a significant impact on the relapse (p value = 0.001). On further analysis; tumor rupture and spillage was a predictor for recurrence reaching a statistical significance (p value = 0.003), whereas the other violations were not.ConclusionsFavorable outcomes could be achieved by compliance with evidence-based guidelines even in a resource-limited country like ours. Violations were correlated with relapse; however, only tumor rupture and spillage was of statistical significance in multivariate analysis. Failure of lymph node documentation was the main problem encountered, and it should be avoidable in future practice.