Stephen J. Schueler, M.D.

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Von Hippel-Lindau Disease Underlying Cause

The cause of von Hippel-Lindau is hereditary. Von Hippel-Lindau disease causes tumors (hemangioblastomas) to form in the kidneys, brain, eye, brainstem, spinal cord, adrenal glands, and pancreas. Those with Von Hippel-Lindau disease have an increased risk for cancer, especially kidney cancer.

Those with von Hippel-Lindau disease have:

  • 15% have tumors of the spinal cord
  • 23% have kidney cancer
  • 30% have tumors of the adrenal gland
  • 50% have abdominal cysts: kidney, pancreas
  • 54% have tumors of the cerebellum
  • 57% have tumors of the retina

Continue to Von Hippel-Lindau Disease Genetics

Last Updated: Nov 24, 2010 References
Authors: Stephen J. Schueler, MD; John H. Beckett, MD; D. Scott Gettings, MD
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PubMed Von Hippel-Lindau Disease References
  1. Joerger M, Koeberle D, Neumann HP, Gillessen S. Von Hippel-Lindau disease--a rare disease important to recognize. Onkologie. 2005 Mar;28(3):159-63. [15772467]
  2. Maher ER. Von Hippel-Lindau disease. Curr Mol Med. 2004 Dec;4(8):833-42. [15579030]
  3. Richard S; French VHL Study Group. von Hippel-Lindau disease: recent advances and therapeutic perspectives. Expert Rev Anticancer Ther. 2003 Apr;3(2):215-33. [12722881]
  4. Slater A, Moore NR, Huson SM. The natural history of cerebellar hemangioblastomas in von Hippel-Lindau disease. AJNR Am J Neuroradiol. 2003 Sep;24(8):1570-4. [13679272]
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