Wilms tumor and associated predisposing syndromes and conditions

Wilms tumor, also known as nephroblastoma, is relatively rare but is the most common renal malignancy in children, accounting for about 5% of all childhood malignancies and 90% of all childhood renal malignancies. Advances in the understanding of the underlying cause and pathophysiology of Wilms tumor have significantly improved the prognosis and survival rates for children with Wilms tumor, but cases may occur sporadically and certain affected patients face higher rates of relapse and morbidity. The most common clinical presentation involves the identification of an abdominal mass or swelling without other signs or symptoms. The nonspecific nature of symptoms that can present can delay timely diagnosis and treatment. Numerous predisposing syndromes are associated with an increased risk for the development of Wilms tumor. Clinicians who recognize these syndromes and other conditions and understand the increased risk can provide the appropriate level of anticipatory guidance and use the optimal screening plan.