Current Recommendations, Controversies, and Potential Novel Approaches in the Treatment of Wilms Tumor

Abstract

Wilms tumor (WT) is one of the most common renal malignancies in children, comprising about 5% of all childhood cancers. If diagnosed early, WT responds well to appropriate interventions such as surgical resection, chemotherapy and radiotherapy, with a 5 year survival higher than 85%. In this review, we will describe the first-line treatment options for WT, discuss controversies related to particular modes of therapy, and highlight promising advances in molecular biology that may serve as effective therapeutics in the near future. Current treatment protocols for WT include nephrectomy followed by postoperative chemotherapy with or without preoperative chemotherapy. Though both are acceptable forms of treatment, preoperative chemotherapy prior to tumor resection has been shown to reduce tumor size and decrease the risk of intraoperative tumor rupture. Preoperative transcatheter arterial chemoembolization has also been shown to improve tumor resections and relapse-free survival rates, potentially providing an additive method to improve WT outcomes. Radical nephrectomy is the mainstay surgical treatment for WT, however evidence suggests that partial nephrectomies may be an equally viable option. Radiotherapy traditionally utilizes the anteroposterior-posteroanterior field technique, but recent advances have allowed for tumor-specific targeting and sparing of non-neoplastic tissues using intensity-modulated radiation therapy and volumetric-modulated arc therapy. Lastly, potential targets for future therapy include the β-catenin pathway, which has been found to be important in the development of WT, in addition to advances in applying microRNA, M6620, and stem cell therapy.