Pheochromocytoma

A pheochromocytoma is a tumor which is usually found in the adrenal gland. It secretes hormones that are similar to, and often include, adrenaline. Adrenaline is also called epinephrine.

What is going on in the body?

A pheochromocytoma tumor is usually found in the adrenal gland, although it may occur in other places. The body has two adrenal glands, which sit on top of the kidneys. The tumor often causes symptoms because of the adrenaline-like hormones it secretes. A pheochromocytoma tends to have sudden releases of hormones, which cause a sudden "attack", or onset of symptoms.

What are the causes and risks of the disease?

The cause of a pheochromocytoma is unknown. Some rare inherited conditions can increase the risk of getting this tumor. These conditions include the MEN syndrome, which is an inherited disease that causes tumors in many glands, and neurofibromatosis, a genetic disorder that affects the growth of nerve tissue.

What are the signs and symptoms of the disease?

Some symptoms, such as high blood pressure, may be constant in a person with a pheochromocytoma. Other symptoms may be noticeable only during the attacks. Symptoms may include:

How is the disease diagnosed?

The history of symptoms and the physical exam will make a healthcare provider suspect a pheochromocytoma. The tumor is often found in people with very high blood pressure. Urine tests are usually done to help confirm the elevated levels of adrenaline-like hormones. An abdominal CT scan or other imaging test is done to locate the tumor.

What can be done to prevent the disease?

There is no known way to prevent a pheochromocytoma.

What are the long-term effects of the disease?

In most people, surgery to remove the pheochromocytoma is successful. In these cases, there are often no long-term effects. In others, however, the tumor may cause death. This may be due to a massive release of hormones by the tumor. Occasionally, the tumor may cause death by spreading to other parts of the body.

What are the risks to others?

A pheochromocytoma is not contagious.

What are the treatments for the disease?

Surgery is the treatment of choice to remove the pheochromocytoma. Medications are used to help control the high blood pressure before and during surgery. Some people cannot tolerate surgery, or surgery cannot remove the entire tumor. In this case, blood pressure medications and chemotherapy may be needed.

What are the side effects of the treatments?

Surgery carries a risk of bleeding, infection, and allergic reactions to anesthesia. The medications used to control blood pressure may cause allergic reactions, stomach upset, and other side effects.

What happens after treatment for the disease?

Most people have the pheochromocytoma removed during surgery and are "cured." These people can usually return to normal activities after recovery. When a person cannot tolerate surgery, or when the tumor cannot be completely removed, death may occur.

How is the disease monitored?

If the pheochromocytoma is successfully removed, urine tests and blood pressure measurements are done for a few years. These tests can help detect a recurrence of the tumor, which allows earlier treatment. Any new or worsening symptoms should be reported to the healthcare provider. A person who needs chemotherapy will require close monitoring with blood and x-ray tests. These help to check if the treatment is working and monitor for side effects.

Author:Bill Harrison, MD
Date Written:
Editor:Duff, Ellen, BA
Edit Date:10/09/00
Reviewer:Adam Brochert, MD
Date Reviewed:07/27/01

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