Kidney cancer affects approximately 30,000 individuals in the United States and is responsible for more than 12,000 deaths each year (1 ,2 ). It has been estimated that 3% of adult malignancies are kidney cancers (1 ). Like many cancers, such as breast and colon cancers, kidney cancer occurs in both sporadic and familial forms. The vast majority of kidney cancer—up to 96%—is sporadic or nonhereditary, while 4% is thought to have a hereditary basis (2 ). There are at least four types of familial kidney cancer that have been categorized: renal carcinoma associated with Birt-Hogg-Dube syndrome, hereditary papillary renal carcinoma, hereditary clear cell renal carcinoma, and renal carcinoma associated with the von Hippel-Lindau (VHL) disease (3 ). This chapter will focus on the recent molecular understanding of VHL disease.
