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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
-
-