Genetic Events in the Development of Wilms' Tumor

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Abstract
Wilms' tumor is the most common intraabdominal solid tumor of childhood, affecting 1 in 10,000 children worldwide. Treatment includes surgical resection and chemotherapy for virtually all affected children and additional radiotherapy for those with advanced disease or adverse prognostic features. This approach leads to cure rates exceeding 80 percent1.Certain features require particular attention in management and also provide a key to understanding the complex genetic pathways involved in the development of this tumor. The occurrence of bilateral Wilms' tumor in approximately 5 to 10 percent of affected children,2 dictating kidney-sparing resection, indicates an underlying genetic predisposition. Another peculiar . . .