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

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

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