Pheochromocytoma

  • Medical Author:
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

  • Medical Editor: Charles Patrick Davis, MD, PhD
    Charles Patrick Davis, MD, PhD

    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

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What are the symptoms of a pheochromocytoma?

Someone with a pheochromocytoma usually has three classic symptoms -- headache, sweating, and heart palpitations (a fast heart beat) in association with markedly elevated blood pressure (hypertension). Other conditions that may accompany these classic symptoms are as follows: anxiety, nausea, tremors, weakness, abdominal pain, and weight loss.

Some people, however, never develop symptoms of a pheochromocytoma. Up to 10% of cases are discovered incidentally, meaning that they are not suspected and only found when the patient is undergoing diagnostic studies for other conditions. In some cases, the high blood pressure comes and goes and may be difficult to document. In other cases, the blood pressure is consistently elevated and easily recorded.

Pheochromocytomas are present in only about 0.2% of all people with high blood pressure. There are certain conditions, however, in which the diagnosis of pheochromocytoma may rank high on the list of possibilities; they are discussed below.

What conditions are associated with pheochromocytomas?

Pheochromocytomas can be a component of certain familial or genetic syndromes. The most common familial condition is called multiple endocrine neoplasia, or MEN for short. Two types of MEN -- MEN 2A and 2B -- are associated with pheochromocytomas. Both are genetic syndromes that run in families and are transmitted from parent to child in an autosomal dominant manner.

Pheochromocytomas are not the only tumors that occur in MEN 2A and 2B. MEN 2A carries an increased risk of tumors of the parathyroids, glands near the thyroid that help to regulate calcium levels in the body. And both MEN 2A and 2B elevate the risk of medullary carcinoma of the thyroid - an unusual type of  thyroid cancer. In families where MEN is suspected, genetic testing can be done to help identify family members at risk.

Pheochromocytomas are a feature of other genetic disorders, including von Hippel-Lindau syndrome and neurofibromatosis type 1. Both of these disorders are associated with the development of numerous benign and malignant tumors.

There are also many individuals who have pheochromocytomas with no known family history of them. These cases are termed sporadic. In general, if these patients have bilateral disease (pheochromocytomas in both adrenal glands) or are diagnosed before the age of 21, genetic screening is recommended.

Medically Reviewed by a Doctor on 3/27/2015
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