von Hippel-Lindau syndrome


Also found in: Acronyms.

von Hip·pel-Lin·dau syn·drome

(făn hip'el lan'dow), [MIM*193300]
a type of phacomatosis, consisting of retinal vascular malformations, which may be multiple and bilateral, associated with hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord; sometimes associated with renal cell carcinomas or cysts or hamartomas of kidney, adrenal, or other organs; autosomal dominant inheritance due to mutation in the von Hippel-Lindau gene (VHL) on 3p.

von Hip·pel-Lin·dau syn·drome

(făn hip'el lan'dow), [MIM*193300]
a type of phacomatosis, consisting of retinal vascular malformations, which may be multiple and bilateral, associated with hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord; sometimes associated with renal cell carcinomas or cysts or hamartomas of kidney, adrenal, or other organs; autosomal dominant inheritance due to mutation in the von Hippel-Lindau gene (VHL) on 3p.

Lindau,

Arvid Wilhelm, Swedish pathologist, 1892-1958.
Lindau disease - Synonym(s): von Hippel-Lindau syndrome
Lindau tumor - a benign cerebellar neoplasm. Synonym(s): hemangioblastoma
von Hippel-Lindau syndrome - see under von Hippel

von Hippel,

Eugen, German ophthalmologist, 1867-1939.
Hippel keratoplasty
Hippel trephine
von Hippel disease - retinal hemangiomatosis.
von Hippel-Lindau syndrome - a type of phacomatosis, consisting of hemangiomas of the retina associated with hemangiomas or hemangioblastomas primarily of the cerebellum and walls of the fourth ventricle, occasionally involving the spinal cord. Synonym(s): cerebroretinal angiomatosis; Lindau disease
References in periodicals archive ?
Hearing preservation surgery for small endolymphatic sac tumors in patients with von Hippel-Lindau syndrome.
Clear cell NETs will be discussed in the section on von Hippel-Lindau syndrome (Figure 3, A).
Transcriptional regulation of phenylethanolamine N-methyltransferase in pheochromocytomas from patients with von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2.
Carcinoid tumor of the common bile duct-a novel complication of von Hippel-Lindau syndrome.
This includes the ocular and central nervous system hemangioblastomas, pheochromocytomas, or pancreatic tumors indicative of Von Hippel-Lindau syndrome and the fibrofolliculomas or pulmonary cysts often seen in Birt-Hogg-Dube syndrome.
21) There were no cases of clear cell carcinoids associated with the von Hippel-Lindau syndrome, as described in the gallbladder (41) and extrahepatic bile ducts.
Two cases of bile duct NET, reported in patients with von Hippel-Lindau disease, may be related to the propensity for patients with von Hippel-Lindau syndrome to develop neuroendocrine tumors.
Papillary cystadenoma of epididymis: component of von Hippel-Lindau syndrome.
Basic criteria for clinical diagnosis and genetic counseling in von Hippel-Lindau syndrome.