What Is Pheochromocytoma?

This is a rare tumor that usually grows in the middle of your lower back in your adrenal glands. It is most common in adults ages 30 to 50, but people of all ages can have it. Children make up about 10% of all cases.

Few pheochromocytoma tumors spread to other organs. But they can be dangerous and should be treated right away.

Your adrenal glands make hormones that control your metabolism, blood pressure, and other important functions. A pheochromocytoma also releases hormones and does so at much higher levels than normal. The hormones made by these tumors cause high blood pressure, which can damage your heart, brain, lungs, and kidneys.

Symptoms

Some people with these tumors have high blood pressure all the time. For others, it goes up and down.

High blood pressure may be your only symptom. But most people have at least one of the following as well:

These symptoms can come on suddenly, like an attack, several times a day. But they also can happen just a few times a month. As the tumor grows, these attacks may become stronger and happen more often.

If you or your child has symptoms like these, see a doctor right away.

Causes

Doctors don’t know why most of these tumors form.

About 30%, however, seem to run in families. These are more likely to be cancerous than ones that appear randomly. They can spread to other parts of your body, including your liver, lungs, or bones.

The tumors are more common in people with inherited disorders or conditions, including:

  • Multiple endocrine neoplasia, type II
  • Von Hippel-Lindau disease
  • Neurofibromatosis 1 (NF1)
  • Hereditary paraganglioma syndrome

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Diagnosis

Many people who have pheochromocytoma are never diagnosed because the symptoms are so much like those of other conditions. But there are ways to find out if you have one of the tumors:

  • Blood or urine tests to tell if you have high levels of hormones in your body
  • An MRI (magnetic resonance imaging), which uses powerful magnets and radio waves to make images of organs and tissues to check for a tumor
  • A CT scan (computed tomography), which puts together several X-rays taken from different angles to see if there’s a tumor.

If you have a pheochromocytoma, your doctor may recommend tests to see if it was caused by a genetic disorder. This can tell you if you’re at risk for more in the future and whether your children and other family members are at higher risk.

A parent with a damaged gene has a 50% chance of passing it to his or her child.

Treatment

You’ll most likely need surgery to remove the tumor. Your surgeon may be able to do this using tiny cuts instead of one large opening. This is called laparoscopic, or minimally invasive, surgery. It can shorten your recovery time.

Before the surgery, you may need to take medication to lower your blood pressure and control an occasional fast heart rate.

If you have a tumor in only one adrenal gland, your surgeon will probably remove that whole gland. Your other gland will still make the hormones your body needs.

If you have tumors in both glands, your surgeon may remove just the tumors and leave part of the glands.

If both glands need to be removed, you can take steroids to help replace the hormones your body won’t be able to make anymore.

If all goes well with the surgery, chances are good that your symptoms will go away and your blood pressure will return to normal.

If your tumor is cancerous, you may also have radiation and chemotherapy to help keep it from growing.

WebMD Medical Reference Reviewed by James Beckerman, MD, FACC on May 09, 2016

Sources

SOURCES:

National Organization for Rare Diseases: “Pheochromocytoma.”

Medscape: “Pheochromocytoma Treatment & Management.”

Johns Hopkins Medicine: “Adrenal Glands.”

UpToDate.com: “Clinical presentation and diagnosis of pheochromocytoma.”

OncoLink (Penn Medicine): “All About Pheochromocytoma.”

Zuber, S. Endocrinology and Metabolism Clinics of North America, June 2012.

Dana Farber Cancer Institute: “Ask the cancer genetics team: inherited tendency for pheochromocytomas.”

University of Maryland Medical Center: “Adrenal Glands.”

Medscape: “Pediatric Pheochromocytoma Treatment & Management.”

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