Renal function in patients with Wilms tumor

• Wilms tumor can be associated with significant renal dysfunction.
• Molecular stratification will assist in identifying patients at higher risk for renal dysfunction.
• The role of nephron-sparing surgery may be expanded beyond bilateral disease in light of newer molecular findings and syndrome definitions.

Survival in patients with Wilms tumor (WT) is excellent compared with other pediatric malignancies and adult renal tumors. Treatment-related long-term morbidity and mortality in WT survivors is an area of increasing concern. Renal dysfunction is an example of one of the most feared long-term issues observed in these survivors. Direct toxicity from chemotherapy and radiation as well as direct nephron ablation from surgical treatment (nephrectomy) renders the kidney susceptible to a multitude of problems over time in patients with WT.In this article, we review the existing literature pertaining to renal function in these patients. Incidence rates, causes, and methods to mitigate renal dysfunction are presented in 3 distinct clinical situations: sporadic unilateral WT, syndromic unilateral WT, and bilateral WT. We also offer a critical lens on the current role of nephron-sparing surgery as a means to preserve renal function in these patients. Finally, we discuss potential avenues for refining renal function preservation in patients with WT in the future. We conclude that: (1) renal function in pediatric cancer survivors must be carefully ascertained prospectively using methods that allow diagnosis of mild cases (rather than focus solely on extreme cases represented by the development of end-stage renal disease), (2) every effort should be made to recognize subtle features of predisposition syndromes to avoid syndromic cases from being misclassified and treated as sporadic, (3) molecular stratification for disease aggressiveness as well as multifocality and renal dysfunction will be very important to tailor treatment and balance survival with preservation of renal function, and (4) the role and potential benefits of nephron-sparing surgery deserves careful exploration under well-designed protocols.