What is 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

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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