Renal cell carcinoma in von Hippel-Lindau disease


  • 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


  • Autosomal dominant condition with an incidence of 1:36,000 to 1:45,000

  • Renal cell carcinomas (RCCs) develop in ~60% of patients.

  • Mean age of RCC onset is 39 (range, 16–67) years, significantly younger than sporadic RCC patients with a mean age of 61 years.

You're Reading a Preview

Become a Clinical Tree membership for Full access and enjoy Unlimited articles

Become membership

If you are a member. Log in here