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Renal cell carcinoma in von Hippel-Lindau disease
Definition
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An autosomal dominant disease caused by germline mutations in the von Hippel-Lindau ( VHL ) tumor suppressor gene on 3p25.3 and characterized by a constellation of tumors including renal cysts and clear cell renal cell carcinoma
Clinical features
Epidemiology
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Autosomal dominant condition with an incidence of 1:36,000 to 1:45,000
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Renal cell carcinomas (RCCs) develop in ~60% of patients.
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Mean age of RCC onset is 39 (range, 16–67) years, significantly younger than sporadic RCC patients with a mean age of 61 years.
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