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$Unique_ID{BRK04104}
$Pretitle{}
$Title{Pheochromocytoma}
$Subject{Pheochromocytoma Chromaffin Cell Tumor Adrenal Tumor Sipple's
Syndrome Neurofibromatosis (von Recklinghausen's Disease) von Hippel-Lindau
Disease}
$Volume{}
$Log{}
Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc.
533:
Pheochromocytoma
** IMPORTANT **
It is possible that the main title of the article (Pheochromocytoma) is
not the name you expected. Please check the synonym list to find the
alternate names and disorder subdivisions covered by this article.
Synonyms
Chromaffin Cell Tumor
Adrenal Tumor
Information on the following diseases can be found in the Related
Disorders section of this report:
Sipple's Syndrome
Neurofibromatosis (von Recklinghausen's Disease)
von Hippel-Lindau Disease
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Pheochromocytoma is a rare disorder caused by tumors in the adrenal
glands. It may or may not be a genetically-caused disorder. It is
characterized by high blood pressure which does not respond to ordinary
treatment.
Symptoms
Major symptoms of Pheochromocytoma may include high blood pressure, rapid
breathing and heart beat, profuse sweating, flushed, cold and clammy skin,
and severe headache. Angina (pain in the heart), palpitations of the heart,
nausea, vomiting, stomach pain, visual disturbances, tingling sensations,
constipation and an abnormal sense of impending doom may also occur.
Causes
Pheochromocytoma is caused by the development of tumors in the adrenal
glands. The tumors may also develop along certain nerve pathways. This
disorder may appear for no apparent reason or in some cases may be inherited
through an autosomal dominant gene with incomplete penetrance. (Human traits
including the classic genetic diseases, are the product of the interaction of
two genes for that condition, one received from the father and one from the
mother. In dominant disorders, a single copy of the disease gene (received
from either the mother or father) will be expressed "dominating" the normal
gene and resulting in appearance of the disease. The risk of transmitting
the disorder from affected parent to offspring is 50% for each pregnancy
regardless of the sex of the resulting child.) Incomplete penetrance means
that all characteristics of a particular trait may not be manifested in all
those who inherit the gene).
Affected Population
Pheochromocytoma affects males and females in equal numbers. People of any
age can be affected, but symptoms usually appear before fifty years of age.
Related Disorders
Symptoms of the following disorders can be similar to those of
Pheochromocytoma. Comparisons may be useful for a differential diagnosis:
Sipple's Syndrome is characterized by adrenal tumors associated with
tumors of other endocrine glands such as thyroid and parathyroid.
Neurofibromatosis (von Recklinghausen Disease) is a genetic disorder with
highly variable manifestations which can affect many body systems. The
disease is characterized by multiple nerve tumors under the skin which can
result in disfigurement, and other complications. (For more information on
this disorder, choose "Neurofibromatosis " as your search term in the Rare
Disease Database).
von Hippel-Lindau Disease is an hereditary disorder characterized by
headache, dizziness and failure of muscular coordination. Uncontrollable
high blood pressure, the presence of tumors in the adrenal glands
(pheochromocytoma) and abnormalities in the blood vessels of the retina may
also occur. ( For more information on this disorder, choose "von Hippel" as
your search term in the Rare Disease Database.)
Therapies: Standard
Diagnosis of Pheochromocytoma may be confirmed through urine testing, lack of
responsiveness to drug treatment of the high blood pressure, and magnetic
resonance imaging (MRI). This fairly new technique has proven superior to
the use of x-rays for soft tissue diagnosis. Treatment of Pheochromocytoma
usually involves the surgical removal of the tumor. Other treatment is
symptomatic and supportive. Genetic counseling may be indicated in some
cases when Pheochromocytoma occurs in families.
Therapies: Investigational
This disease entry is based upon medical information available through
October 1989. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Pheochromocytoma, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
The National Adrenal Diseases Foundation
505 Northern Blvd., Suite 200
Great Neck, NY 11021
(516) 487-4992
American Cancer Society
1599 Clifton Rd., NE
Atlanta, GA 30329
(404) 320-3333
NIH/National Cancer Institute
9000 Rockville Pike, Bldg. 31, Rm. 1A2A
Bethesda, MD 20892
1-800-4-CANCER
The National Cancer Institute has developed PDQ (Physician Data Query), a
computerized database designed to give doctors quick and easy access to many
types of information vital to treating patients with this and many other
types of cancer. To gain access to this service, a doctor can contact the
Cancer Information Service offices at 1-800-4-CANCER. Information
specialists at this toll-free number can answer questions about cancer
prevention, diagnosis, and treatment.
For genetic information and genetic counseling referrals:
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
(914) 428-7100
Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD 20815
(800) 336-GENE
(301) 652-5553
References
LOCALIZATION OF ECTOPIC PHEOCHROMOCYTOMAS BY MAGNETIC RESONANCE IMAGING: J.E.
Schmedt, et al.; Am J Med ( October, 1987: issue 83 (4)). Pp. 770-772.
CLINICALLY UNSUSPECTED PHEOCHROMOCYTOMAS. EXPERIENCE AT HENRY FORD
HOSPITAL AND A REVIEW OF THE LITERATURE: N.K. Krane; Arch Intern Med
(January, 1986: (issue 146 (1)). Pp. 54-57.
DIAGNOSIS AND MANAGEMENT OF PHEOCHROMOCYTOMA: G.W. Gifford, et al.;
Cardiology (1985; 72 Suppl. 1). Pp. 126-130.
INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown
and Co., 1987. Pp. 557-559, 1962-1966, 2302