Article title: NINDS von Hippel-Lindau Disease Information Page: NINDS
Main condition: von Hippel-Lindau disease
Conditions: von Hippel-Lindau disease
Table of Contents (click to jump to sections)What
is von Hippel-Lindau disease (VHL)?
Is
there any treatment?
What
is the prognosis?
What
research is being done?
Selected
references
Organizations
What is von
Hippel-Lindau disease (VHL)?
von Hippel-Lindau disease (VHL) is a
rare, genetic multi-system disorder characterized by the abnormal growth
of tumors in certain parts of the body (angiomatosis). The tumors of the
central nervous system (CNS) are benign and are comprised of a nest of
blood vessels and are called hemangioblastomas (or angiomas in the eye).
Hemangioblastomas may develop in the brain, the retina of the eyes, and
other areas of the nervous system. Other types of tumors develop in the
adrenal glands, the kidneys, or the pancreas.
Symptoms of VHL vary among
patients and depend on the size and location of the tumors. Symptoms may
include headaches, problems with balance and walking, dizziness, weakness
of the limbs, vision problems, and high blood pressure. Cysts
(fluid-filled sacs) and/or tumors (benign or cancerous) may develop around
the angiomas and cause the symptoms listed above. Individuals with VHL are
also at a higher risk than normal for certain types of cancer, especially
kidney cancer.
Is there any
treatment?
Treatment for VHL varies according to the location and
size of the angiomas. In general, the objective of treatment is to treat
the growths when they are small so that they do not cause permanent
problems by putting pressure on the brain or spine, blocking the flow of
cerebrospinal fluid in the nervous system, or impairing vision. Treatment
of most cases of VHL usually involves surgery to remove the tumors before
they become harmful. Certain tumors can be treated with focused high-dose
irradiation. Individuals with VHL need careful monitoring by a physician
and/or medical team familiar with the disorder.
What is the prognosis?
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.
What research is being
done?
The NINDS pursues a vigorous program of research aimed at
preventing and treating disorders that cause tumors in the brain and
spinal cord such as VHL.
Selected references
Bradley, W, et al (eds).
Neurology in Clinical Practice:
Principles of Diagnosis and Management, vol. II Butterworth-Heinemann,
Boston, pp. 1332-1333 (1991)
Joynt, R (ed.)
Clinical Neurology, vol. 3 J.B. Lippincott
Co., Philadelphia, pp. 7, 39, 83 (1992)
Karsdorp, N, et al.
von Hippel-Lindau Disease: New Strategies
in Early Detection and Treatment. The American Journal of Medicine 97;
158-168 (August 1994)
Lesho, E.
Recognition and Management of von Hippel-Lindau
Disease. American Family Physician 50:6; 1269-1272 (November 1, 1994)
Martz, C.
von Hippel-Lindau Disease: A Genetic Condition
Predisposing Tumor Formation. Oncology Nursing Forum 18:3; 545-551 (1991)
Genetic Alliance
4301 Connecticut Avenue, N.W.
Suite 404
Washington, DC 20008-2304
info@geneticalliance.org
http://www.geneticalliance.org/
Tel:
202-966-5557 800 336-GENE (4363)
Fax: 202-966-8553
National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel:
203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481
Von Hippel Lindau Family Alliance
171 Clinton Road
Brookline, MA 02445
info@vhl.org
http://www.vhl.org/
Tel: 617-277-5667
800-767-4VHL (4845)
Fax: 617-734-8233
This fact sheet is in the public domain. You may copy it.Provided
by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD
20892
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