Wilms tumor: Progress and considerations for the surgeon

Abstract

Wilms tumor (WT) or nephroblastoma is the most common tumor of renal origin found in children. It accounts for 6% of all pediatric tumors and is the second most frequent intrabdominal solid organ tumor found in children. Initial survival rates in the early part of the last century was only 30%, but now long-term survival in both North America and European trials is approaching 85% with many low-stage tumors significantly higher. Treatment is now progressing towards "risk-based management"- based not only on stage and histology but also incorporating genetic markers [Dome JS, Grundy PE, Perlman EJ, Ehrlich PF, et al. Protocols for the renal tumors study. Childrens Oncology Group. [www.childrensoncologygroup.org. 2007.]. Within the multidisciplinary treatment team the surgeon plays a critical role in the diagnosis, staging and the surgeon's technical skills and judgment directs therapy and impacts outcome. The next generation of treatment for children with WT will focus on identifying subsets of patients who can be defined by some criterion as having a different outcome than their similar stage peers and who therefore require a variation in management. These include children with WT that have unsatisfactory long-term survival (less then 75%), patients of good survival but high potential for late effects and a final challenge are those children with both a poor survival and a high potential for late effects. This article presents a review of the most recent treatment considerations for WT with a focus on the surgeon's role to ensure a good outcome.