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What Is Pheochromocytoma?

Humans have two adrenal glands located above the kidneys. They make substances called hormones that regulate blood pressure, fluid metabolism, and other body functions. A pheochromocytoma is an uncommon tumor of the adrenal gland. Rarely, this type of tumor develops outside the adrenal glands.

Pheochromocytomas secrete a hormone called epinephrine or related compounds. These substances can cause high blood pressure, heart palpitations, headaches, and sweating. Pheochromocytomas account for a very small number of hypertension cases.

What Causes Pheochromocytoma?

The cause is unknown. Most tumors aren’t related to family history, but about 10% are part of familial or hereditary endocrine tumor disorders.

What Are the Symptoms of Pheochromocytoma?

Common symptoms are headaches that come and go, anxiety, palpitations (abnormal, rapid heartbeats), sweating, high blood pressure, and heat intolerance.

How Is Pheochromocytoma Diagnosed?

Your health care provider may suspect a pheochromocytoma because of your medical history and physical examination. Urine and blood tests will be done to measure hormone levels. One urine test, a 24-hour urine collection, measures substances called catecholamines. You shouldn’t drink alcohol or caffeine or take amphetamines, benzodiazepines, certain antidepressants, or lithium when doing the test. These substances may lead to false high levels.

Magnetic resonance imaging (MRI), CT, and other scans may be done to look for the tumor. Pheochromocytomas located outside the adrenals may need whole-body imaging with special nuclear medicine tests to be found.

How Is Pheochromocytoma Treated?

More than 90% of pheochromocytomas are in the adrenal glands and can be cured with surgery. Medicines to control blood pressure should be given before surgery.

Temporary low and high blood pressure readings can occur while the tumor is being removed.

Pheochromocytomas that are malignant and have spread cannot be cured with surgery. A combination of chemotherapy, radiation therapy, and other treatments is used to help control the disease.

DOs and DON’Ts in Managing Pheochromocytoma:

  • DO tell your health care provider if you had pheochromocytomas before or family members have endocrine tumors. Your family may need screening blood or urine tests.
  • DO call your health care provider if you have vision changes, severe headache, weakness on one side of the body, chest pains, or increasing palpitations.
  • DO call your health care provider if you have ankle swelling, shortness of breath, or weakness or dizziness when standing.
  • DO call your health care provider if symptoms return after surgery.
  • DON’T do strenuous exercise until your pheochromocytoma has been removed.
  • DON’T expect that your high blood pressure will be completely normal after the operation. Some permanent changes may have already occurred in the kidneys and blood vessels.
FOR MORE INFORMATION

Contact the following sources:

  • National Adrenal Diseases Foundation
    Tel: (516) 487-4992
    Website: http://www.nadf.us/
  • The Endocrine Society
    Tel: (888) 363-6274
    Website: http://www.endo-society.org

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

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