Other Malignant Neoplasms of the Kidney Including Metastatic Lesions

Abstract

Wilms tumor (WT) is the most common pediatric kidney tumor. Ninety-eight percent of WT are diagnosed under 10 years of age; adult instances are rare. The most common presentation is an abdominal mass. WT may be sporadic or syndrome associated. It is unique among renal parenchymal neoplasms because there is a known precursor lesion, i.e., nephrogenic rests. The pathogenesis of WT is related to consistent genetic findings in WT1 at 11p13 and WT2 at 11p15. The WT1 gene and its transcription factors are responsible for normal renal, gonadal, and cardiac development. Germline mutations of WT1 are found in patients with Denys-Drash and Wilms tumor-aniridia-genitourinary malformation-mental retardation (WAGR) syndromes. The WT2 gene mutation is found in a large proportion of sporadic Wilms tumors and also as a part of Beckwith-Wiedemann syndrome. The ontogeny of WT and its complexity has been recently studied and a new revised model with therapeutic and prognostic implications has been proposed.