Wilms' Tumor is the most common pediatric cancer of the kidney (4% of all pediatric cancer cases, 93% of pediatric kidney cancers) which arises from nephroblasts - cells involved in prenatal kidney development. It typically appears before age five, and manifests as a mass within the kidney. Signs include abdominal pain, bloody urine and raised blood pressure. Treatment generally consists of removal of the affected kidney, preceded or followed by chemotherapy and localized radiotherapy.

Susceptibility to Wilms' Tumor seems to be heritable, though the genetic basis is poorly understood. A gene, WT1, which is linked to some cases, appears to be involved in urogenital and kidney development, and also may have a role in the development of prostate cancer.

Sources: http://www.hc-sc.gc.ca/hpb/lcdc/bc/children/other6_e.html (Health Canada), http://www.cancerbacup.org.uk/info/child-wilms.htm (CancerBACUP.org.uk), and PubMed searches for "Wilms tumor" and "WT1".

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