What is the prevalence of renal cell carcinoma in von Hippel-Lindau (VHL) syndrome (disease)?

Updated: May 21, 2019
  • Author: Kush Sachdeva, MD; Chief Editor: E Jason Abel, MD  more...
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Answer

Renal cell carcinoma develops in nearly 40% of patients with von Hippel-Lindau disease and is a major cause of death in these patients. Deletions of chromosome 3p occur commonly in renal cell carcinoma associated with von Hippel-Lindau disease. Chromosome 3p contains several of the genes associated with kidney cancer, including BAP-1 and PBRM-1, among others.


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