This week's artwork was donated by Lonny, a pediatric cancer patient who received treatment at The Children's Hospital of Philadelphia . This review summarizes information on inherited epithelial tumors of the kidney. Emphasis is placed on identifying clinically distinct inherited forms of renal cancer because each distinct clinical syndrome defines a different renal cancer susceptibility gene. So far, two genes that predispose to epithelial cancers of the kidney have been identified, VHL and the MET proto-oncogene.
Available evidence suggests that several renal cancer genes remain to be identified.
Copyright 2000 Academic Press. The VHL disease gene is a putative tumor suppressor gene responsible for VHL disease. Germline missense mutations are clustered in 4 regions in the VHL gene. Since almost of germline mutations are detectable, presymptomatic diagnosis of the VHL disease with DNA analyses is important to improve the prognosis of VHL disease. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.