Wilms tumor is a common kidney tumor in children that comprises three cell types blastemal, stromal, and epithelial cells. It occurs predominantly unilateral, but also bilateral and multifocal.
Wilms tumors account for about 6% of childhood malignancies. Girls are slightly more frequently affected. The total incidence is 7 cases per million children younger than 15 years.
The tumor itself is best diagnosed by ultrasound but congenital genitourinary malformations, hermaphroditism or kidney dysfunction should raise awareness of a tumor disposition and may encourage genetic diagnosis.
This disease is partly caused by congenital syndromes due to germline mutations of the WT1 oder WT2. Other gene possibly involved are p53, FWT1, or FWT2.
The important role of the WT1 gene in tomorgenesis illustrates the fact that in some tumors somatic mutations can be identified. Also patients with heterozygous germline mutations often show homozygous or compound heterozygous mutations in their tumor cells, providing support for a second hit hypothesis.
Zuppan CW et al. (1988) Anaplasia in unilateral Wilms' tumor: a report from the National Wilms' Tumor Study Pathology Center.[^]
Coppes MJ et al. (1994) Genetic events in the development of Wilms' tumor.[^]
Grovas A et al. (1997) The National Cancer Data Base report on patterns of childhood cancers in the United States.[^]