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Von Hippel-Lindau disease

Disease definition

Von Hippel-Lindau disease (VHL) is a familial cancer predisposition syndrome associated with a variety of malignant and benign neoplasms, most frequently retinal, cerebellar, and spinal hemangioblastoma, renal cell carcinoma (RCC), and pheochromocytoma.

ORPHA:892

  • Synonym(s):
    • Familial cerebelloretinal angiomatosis
    • Lindau disease
    • VHL
    • Von Hippel-Lindau syndrome
  • Prevalence: 1-9 / 100 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adult
  • ICD-10: Q85.8
  • OMIM: 193300
  • UMLS: C0019562
  • MeSH: D006623
  • GARD: 7855
  • MedDRA: 10047716

Detailed information

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