Annual incidence in the United States is about 62,000 cases with 14,000 deaths. Cigarette smoking accounts for 20-30% of cases. Risk is increased in acquired renal cystic disease. There are two familial forms: a rare autosomal dominant syndrome and von Hippel-Lindau disease. About 35% of pts with von Hippel-Lindau disease develop renal cancer. Incidence is also increased in those with tuberous sclerosis and polycystic kidney disease.