Abstract
Wilms tumor is a malignant renal tumor comprising 85%–90% of all renal tumors in children under 15years of age. It is bilateral in 5%–8% of cases and familial in 1%–2% of cases. In a significant number of cases (9%–17%) it is associated with congenital anomalies, syndromes or conditions which predispose to Wilms tumor. Genes identified in Wilms tumor include WT1 gene (on chromosome 11p13, mutated in ~15% of cases), CTNNB1 gene (mutated in ~15% of cases), and WTX gene (mutated or deleted in ~18% of cases). Histologically, Wilms tumor consists of three components: blastemal, epithelial, and stromal. These may be present in any proportion, resulting in a large number of histological patterns. Prognosis depends on histology, stage, and some biological markers, but is in general excellent, with over 90% overall survival.
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