Prognostic Factors for Wilms Tumor Recurrence: A Review of the Literature

Simple Summary: A Wilms tumor is a childhood kidney tumor. In high-income countries, 90% of patients with this tumor survive. However, the tumor recurs in 15% of patients. It is important to identify the patients at risk of recurrence in order to adjust treatment in such a way that recurrence may potentially be prevented. However, we are currently unable to determine precisely which patients are at risk of recurrence. Therefore, we present an overview of factors that influence the risk of recurrence, also known as prognostic factors. These factors range from patient-, tumor- and treatment-related characteristics to geographic and socioeconomic factors. In addition to these factors, biological markers, such as genetic alterations, should be studied more intensively as these markers may be able to better identify patients at risk of tumor recurrence. Abstract: In high-income countries, the overall survival of children with Wilms tumors (WT) is similar to 90%. However, overall, 15% of patients experience tumor recurrence. The adverse prognostic factors currently used for risk stratification (advanced stage, high risk histology, and combined loss of heterozygosity at 1p and 16q in chemotherapy-naive WTs) are present in only one third of these cases, and the significance of these factors is prone to change with advancing knowledge and improved treatment regimens. Therefore, we present a comprehensive, updated overview of the published prognostic variables for WT recurrence, ranging from patient-, tumor- and treatment-related characteristics to geographic and socioeconomic factors. Improved first-line treatment regimens based on clinicopathological characteristics and advancing knowledge on copy number variations unveil the importance of further investigating the significance of biological markers for WT recurrence in international collaborations.