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What is Von Hippel-Lindau Disease?

What is Von Hippel-Lindau Disease?

  • Von Hippel-Lindau Disease: Genetic disease causing multiple benign tumors.
  • Von Hippel-Lindau Disease: An inherited familial cancer syndrome which is characterized by development of capillary hemangioblastomas of the central nervous system and retina; clear cell renal carcinoma; pheochromocytoma; pancreatic tumors; and inner ear tumors. The syndrome is associated with germline mutations of the VHL tumor suppressor gene, located on chromosome 3p25-26. Symptoms of VHL syndrome may not be apparent until the third decade of life. CNS hemangioblastoma is the most common cause of death, followed by clear cell renal cell carcinoma. --2004
    Source - Diseases Database

Von Hippel-Lindau Disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Von Hippel-Lindau Disease, or a subtype of Von Hippel-Lindau Disease, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Von Hippel-Lindau Disease as a "rare disease".
Source - Orphanet

Von Hippel-Lindau Disease: Introduction

Types of Von Hippel-Lindau Disease:

Broader types of Von Hippel-Lindau Disease:

How many people get Von Hippel-Lindau Disease?

Prevalance of Von Hippel-Lindau Disease: rare

How serious is Von Hippel-Lindau Disease?

Complications of Von Hippel-Lindau Disease: see complications of Von Hippel-Lindau Disease
Prognosis of Von Hippel-Lindau Disease: The prognosis for patients with VHL depends on the location and complications of the tumors. Untreated, VHL may result in blindness and/or permanent brain damage. With early detection and treatment the prognosis is significantly improved. Death is usually caused by complications of brain tumors or kidney cancer. (Source: excerpt from NINDS von Hippel-Lindau Disease Information Page: NINDS)

What causes Von Hippel-Lindau Disease?

Causes of Von Hippel-Lindau Disease: see causes of Von Hippel-Lindau Disease

What are the symptoms of Von Hippel-Lindau Disease?

Symptoms of Von Hippel-Lindau Disease: see symptoms of Von Hippel-Lindau Disease

Complications of Von Hippel-Lindau Disease: see complications of Von Hippel-Lindau Disease

Can anyone else get Von Hippel-Lindau Disease?

Inheritance: see inheritance of Von Hippel-Lindau Disease

Inheritance of genetic conditions: see details in inheritance of genetic diseases.

Von Hippel-Lindau Disease: Testing

Diagnostic testing: see tests for Von Hippel-Lindau Disease.

Misdiagnosis: see misdiagnosis and Von Hippel-Lindau Disease.

How is it treated?

Treatments for Von Hippel-Lindau Disease: see treatments for Von Hippel-Lindau Disease
Research for Von Hippel-Lindau Disease: see research for Von Hippel-Lindau Disease

Name and Aliases of Von Hippel-Lindau Disease

Main name of condition: Von Hippel-Lindau Disease

Class of Condition for Von Hippel-Lindau Disease: genetic autosomal dominant, cancer

Other names or spellings for Von Hippel-Lindau Disease:

VHL, Angiomatosis, Hippel-Lindau Disease

Cerebroretinal angiomatosis, Familial cerebelloretinal angiomatosis Source - Diseases Database

VHL, VHL
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Research the causes of these diseases that are similar to, or related to, Von Hippel-Lindau Disease:


 » Next page: Prevalence and Incidence of Von Hippel-Lindau Disease

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