Medical Author: Ruchi Mathur, M.D.
Medical Editor: Barbara K. Hecht, Ph.D.

• Introduction
• What is a pheochromocytoma?
• What are the symptoms of a pheochromocytoma?
• What conditions are associated with pheochromocytomas?
• What else can cause the symptoms of a pheochromocytoma?
• How do you make the diagnosis of a pheochromocytoma?
• How is a pheochromocytoma treated?
• What is the outlook with a pheochromocytoma?

Introduction

Pheochromocytoma is a very long word, even for someone who speaks “medical language.” If you happen to be a fan of TV dramas involving young confused doctors, you may have heard the word a few times. It is made up of four parts: pheo-chromo-cyt-oma.

If you are one of the few people who have had a personal experience or know someone who has been suspected of having or has actually had a pheochromocytoma, I hope that this article helps explain what this big word actually means to patients and their families.

What is a pheochromocytoma?

First, the basics and bit of anatomy is necessary to explain pheochromocytoma. Pheochromocytomas are tumors of the adrenal glands. As the name implies the ad-renal glands are located near the “renal” area. In other words, the adrenal glands are little glands that basically sit on top of the kidneys. One adrenal gland sits on top of each of the two kidneys.

Despite their small size, the adrenal glands do big things. They are complex endocrine (hormone secreting) glands. Depending on which area you focus on inside them, you find cells that do different things. There is an area where the cells secrete cortisol, a hormone similar to cortisone. There is another area where cells secrete a hormone called aldosterone which helps in water regulation.

And there is yet another area where the cells secrete substances called catecholamines -- epinephrine, norepinephrine and dopamine. These are “flight or fight” hormones. They are responsible in part for that feeling of an “ad-renal-ine” rush you feel when you are afraid. It is these cells that are involved in a pheochromocytoma. Basically, these cells secrete too much epinephrine, norepinephrine and dopamine, and that causes the clinical signs and symptoms we will discuss below.

Pheochromocytomas are, fortunately, quite rare and the vast majority of them are entirely benign. Only very rarely is a pheochromocytoma malignant.

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