Learn About Von Hippel-Lindau Syndrome

What is the definition of Von Hippel-Lindau Syndrome?

Von Hippel-Lindau syndrome is an inherited disorder characterized by the formation of tumors and fluid-filled sacs (cysts) in many different parts of the body. Tumors may be either noncancerous or cancerous and most frequently appear during young adulthood; however, the signs and symptoms of von Hippel-Lindau syndrome can occur throughout life.

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What are the causes of Von Hippel-Lindau Syndrome?

Mutations in the VHL gene cause von Hippel-Lindau syndrome. The VHL gene is a tumor suppressor gene, which means it keeps cells from growing and dividing too rapidly or in an uncontrolled way. Mutations in this gene prevent production of the VHL protein or lead to the production of an abnormal version of the protein. An altered or missing VHL protein cannot effectively regulate cell survival and division. As a result, cells grow and divide uncontrollably to form the tumors and cysts that are characteristic of von Hippel-Lindau syndrome.

How prevalent is Von Hippel-Lindau Syndrome?

The incidence of von Hippel-Lindau syndrome is estimated to be 1 in 36,000 individuals.

Is Von Hippel-Lindau Syndrome an inherited disorder?

Mutations in the VHL gene are inherited in an autosomal dominant pattern, which means that one copy of the altered gene in each cell is sufficient to increase the risk of developing tumors and cysts. Most people with von Hippel-Lindau syndrome inherit an altered copy of the gene from an affected parent. In about 20 percent of cases, however, the altered gene is the result of a new mutation that occurred during the formation of reproductive cells (eggs or sperm) or very early in development.

Who are the top Von Hippel-Lindau Syndrome Local Doctors?
Elite
Highly rated in
38
conditions
Neurosurgery

Ohio State University Wexner Medical Center

Ohio State Brain And Spine Hospital

300 W 10th Ave 
Columbus, OH 43210

Russell Lonser is a Neurosurgery doctor in Columbus, Ohio. Dr. Lonser has been practicing medicine for over 28 years and is rated as an Elite doctor by MediFind in the treatment of Von Hippel-Lindau Syndrome. He is also highly rated in 38 other conditions, according to our data. His top areas of expertise are Von Hippel-Lindau Syndrome, Hemangioblastoma, Hemangioma, and Meningioma. He is licensed to treat patients in Ohio and California. Dr. Lonser is currently accepting new patients.

Elite
Highly rated in
35
conditions

University Of Cambridge

Nihr Cambridge Biomedical Research Centre, Cancer Research Uk Cambridge Centre 
Cambridge, ENG, GB CB20S

Eamonn Maher is in Cambridge, United Kingdom. Maher is rated as an Elite expert by MediFind in the treatment of Von Hippel-Lindau Syndrome. He is also highly rated in 35 other conditions, according to our data. His top areas of expertise are Von Hippel-Lindau Syndrome, Pheochromocytoma, Beckwith-Wiedemann Syndrome, and Macroglossia.

 
 
 
 
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Elite
Highly rated in
46
conditions
Neurosurgery

UVA Health System

Emily Couric Clinical Cancer Center

1240 Lee St 
Charlottesvle, VA 22908

Ashok Asthagiri is a Neurosurgery doctor in Charlottesvle, Virginia. Dr. Asthagiri has been practicing medicine for over 21 years and is rated as an Elite doctor by MediFind in the treatment of Von Hippel-Lindau Syndrome. He is also highly rated in 46 other conditions, according to our data. His top areas of expertise are Von Hippel-Lindau Syndrome, Neurofibromatosis Type 2, Schwannoma, and Neurofibromatosis. He is licensed to treat patients in Virginia. Dr. Asthagiri is currently accepting new patients.

What are the latest Von Hippel-Lindau Syndrome Clinical Trials?
Electromagnetic Tracking of Devices During Interventional Procedures
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MyVHL: Patient Natural History Study
Who are the sources who wrote this article ?

Published Date:updated Last, October

Published By: National Institutes of Health

What are the Latest Advances for Von Hippel-Lindau Syndrome?
Suprasellar paraganglioma in a clinical setting of von Hippel-Lindau syndrome.
Effective Balloon Pulmonary Angioplasty in a Patient with Chronic Thromboembolic Complications after Ventriculoatrial Shunt for Hydrocephalus in von Hippel-Lindau Disease.
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Identification of the feature of immune cells infiltration in inherited renal carcinoma with von Hippel-Lindau syndrome.