What is von Hippel-Lindau (VHL) syndrome (disease)?

Updated: Feb 19, 2021
  • Author: Kush Sachdeva, MD; Chief Editor: E Jason Abel, MD  more...
  • Print
Answer

von Hippel-Lindau syndrome, or von Hippel-Lindau disease, is an autosomal dominant syndrome that confers predisposition to a variety of neoplasms, including the following:

  • Renal cell carcinoma with clear cell histologic features
  • Pancreatic cysts and islet cell tumors
  • Retinal angiomas
  • Central nervous system (CNS) hemangioblastomas
  • Endolymphatic sac tumors
  • Epididymal cystadenomas

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!