Pheochromocytoma

What Is a Pheochromocytoma?

A pheochromocytoma (PCC) is a rare tumor that usually grows in your adrenal glands, above your kidneys. It’s also known as an adrenal paraganglioma or a chromaffin cell tumor.

It’s most common in adults ages 30 to 50, but people of all ages can have it. Children make up about 10% of all cases.

Your adrenal glands make hormones that control things like your metabolism and blood pressure. A pheochromocytoma also releases hormones, at much higher levels than usual. These extra hormones cause high blood pressure, which can damage your heart, brain, lungs, and kidneys.

About 10% to 15% of these tumors are cancerous, and they can spread to other parts of your body. But most pheochromocytoma tumors are benign, meaning they’re not cancerous.

Pheochromocytoma Symptoms

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

That may be your only sign of a problem. But most people have at least one other symptom, including:

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

Pheochromocytoma Triggers

Some people’s attacks are set off by things like:

Pheochromocytoma Causes and Risk Factors

Doctors don’t know why most PCC tumors form.

About 30% of cases seem to run in families. These are more likely to be cancerous than ones that appear at random.

The tumors are more common in people who have disorders or conditions that are passed down from parents to children, including:

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

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Pheochromocytoma Complications

If you don’t get treatment, PCC can lead to life-threatening conditions, including:

Pheochromocytoma Diagnosis

Many people who have pheochromocytoma are never diagnosed because the symptoms are so much like those of other conditions. Some tests can tell your doctor if you have it:

  • Blood or urine tests to look for high levels of hormones
  • An MRI (magnetic resonance imaging) scan, which uses powerful magnets and radio waves to make images of organs and tissues to check for a tumor
  • A CT scan, which puts together several X-rays taken from different angles to spot a tumor

If you have PCC, you might have tests to see if it was caused by a problem with your genes. This can tell you if you’re more likely to get another tumor and whether your family members are at higher risk.

Pheochromocytoma Treatment

You’ll most likely need surgery to remove the tumor. Your doctor may be able to do this using tiny cuts instead of one large opening. This is called laparoscopic or minimally invasive surgery. You generally recover faster from those procedures than from traditional surgery.

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 doctor will probably remove that whole gland. The other gland will 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 your tumor is cancerous, you may also have radiation, chemotherapy, or targeted therapy (using drugs to attack specific cancer cells) to help keep it from growing.

Pheochromocytoma Prognosis

If your doctor removes both of your adrenal glands, you can take medication to help replace the hormones that your body won’t be able to make anymore.

Whatever your treatment, you’ll need regular checkups with your doctor to be sure that PCC doesn’t come back. They can also help you manage any long-term side effects of treatment.

According to estimates, about 95% of people diagnosed with a cancerous form of pheochromocytoma that hasn’t spread to other parts of their body live at least 5 more years. If the tumors have spread or come back after treatment, about 50% to 60% of people live at least 5 years after diagnosis.

WebMD Medical Reference Reviewed by Laura J. Martin, MD on February 15, 2020

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.”

Cleveland Clinic: “Pheochromocytoma.”

Merck Manual Consumer Version: “Pheochromocytoma.”

National Cancer Institute: “Pheochromocytoma and Paraganglioma Treatment (PDQ) -- Patient Version.”

Mayo Clinic: “Pheochromocytoma.”

American Society of Clinical Oncology: “Pheochromocytoma and Paraganglioma Guide.”

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