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von Hippel-Lindau Disease MeSH Descriptor Data 2025
An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include HEMANGIOBLASTOMA in the RETINA; CEREBELLUM; and SPINAL CORD; PHEOCHROMOCYTOMA; pancreatic tumors; and renal cell carcinoma (see CARCINOMA, RENAL CELL). Common clinical signs include HYPERTENSION and neurological dysfunctions.
Entry Version
VON HIPPEL LINDAU DIS
Entry Term(s)
Angiomatosis Retinae
Cerebelloretinal Angiomatosis, Familial
Familial Cerebello-Retinal Angiomatosis
Hippel-Lindau Disease
Lindau Disease
Lindau's Disease
VHL Syndrome
von Hippel-Lindau Syndrome
NLM Classification #
QS 677
Public MeSH Note
2008; see HIPPEL-LINDAU DISEASE 1991-2007; see ANGIOMATOSIS 1975-1990
An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include HEMANGIOBLASTOMA in the RETINA; CEREBELLUM; and SPINAL CORD; PHEOCHROMOCYTOMA; pancreatic tumors; and renal cell carcinoma (see CARCINOMA, RENAL CELL). Common clinical signs include HYPERTENSION and neurological dysfunctions.