Identification of the von Hippel-Lindau (VHL) Gene:  Its Role in Renal Cancer

W. Marston Linehan, MD; Michael I. Lerman, MD, PhD; Berton Zbar, MD
JAMA. 1995;273(7):564-570. doi:10.1001/jama.1995.03520310062031.
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RENAL carcinoma, the most common cancer of the kidney, occurs in over 27000 individuals in the United States each year and is responsible for over 11000 deaths annually.1 The incidence of renal carcinoma worldwide has been increasing at an annual rate of approximately 2%.1 Although renal carcinoma has been reported in children as young as 3 years, it most commonly occurs in adults between the ages of 50 and 70 years.2 Renal carcinoma affects males twice as frequently as females and accounts for approximately 3% of adult cancers. Although a number of hormonal, environmental, cellular, and genetic factors have been evaluated, little is known about the etiology of renal cancer.3 There is an increased incidence of renal cancer among workers exposed to asbestos and among leather workers,4,5 and a strong correlation has been found with cigarette smoking.6,7 In patients with end-stage renal disease, particularly


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