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Pheochromocytoma

Alternate Names

  • pheochromoblastoma

Definition

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 release hormones in sudden bursts, which cause "attacks", or waves of symptoms.

Risks

What are the causes and risks of the disease?

The cause of a pheochromocytoma is unknown. Some unusual inherited conditions can increase the risk of getting this tumor. These conditions include the MEN syndrome, a disease characterized by a certain combination of tumors in different glands, and neurofibromatosis, a genetic disorder that affects the growth of nerve tissue.

Prevention

What can be done to prevent the disease?

There is no known way to prevent a pheochromocytoma.

Diagnosed

How is the disease diagnosed?

The history of symptoms may make a healthcare professional suspect a pheochromocytoma.
In a medical evaluation for high blood pressure, a urine test is sometimes done to look for the rare pheochromocytoma among other, more common causes of high blood pressure.
If a 24-hour urine has elevated levels of adrenaline-like hormones, abdominal CT scans, other imaging tests, and further lab tests can be used to confirm the diagnosis.

Long Term Effects

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.

Other Risks

What are the risks to others?

A pheochromocytoma is not contagious.

Treatments

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, and sometimes the surgeon finds it technically impossible to remove the entire tumor. In this case, blood pressure medications and chemotherapy may be needed.

Side Effects

What are the side effects of the treatments?

Surgery can be complicated by bleeding, infection, or an allergic reactions to the anesthetic. The medications used to control blood pressure may cause allergic reactions, stomach upset, and other side effects.

After Treatment

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.

Monitor

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 professional. A person who needs chemotherapy will require close monitoring with blood and x-ray tests to verify that the treatment is working and to monitor for side effects.

Sources

Harrison's Principles of Internal Medicine, 1998, Fauci et al.

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