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Pheochromocytoma

Overview

A pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare, usually noncancerous (benign) tumor that develops in an adrenal gland. Usually, this type of tumor affects one of your two adrenal glands, but it can affect both.

If you have a pheochromocytoma, the tumor releases hormones that cause either episodic or persistent high blood pressure. Untreated, a pheochromocytoma can result in severe or life-threatening damage to other body systems, especially the cardiovascular system.

Most people with a pheochromocytoma are between the ages of 20 and 50, but the tumor can develop at any age. Surgical treatment to remove a pheochromocytoma usually returns blood pressure to normal.

Pheochromocytoma and irregular blood pressure

A pheochromocytoma can cause wild fluctuations in blood pressure with normal blood pressure in between spells. This can make the condition more difficult to detect. The graph shows a nine-day period of short, irregular bursts in blood pressure due to a pheochromocytoma. The lower points represent the bottom number of the reading (diastolic pressure). The higher points show the top number of the reading (systolic pressure). For example, the first burst in blood pressure is seen on day two with a reading of 250/110 millimeters of mercury.

Symptoms

Signs and symptoms of pheochromocytomas often include:

  • High blood pressure
  • Heavy sweating
  • Headache
  • Rapid heartbeat (tachycardia)
  • Tremors
  • Paleness in the face (pallor)
  • Shortness of breath (dyspnea)

Less common signs or symptoms may include:

  • Anxiety or sense of doom
  • Constipation
  • Weight loss

Triggers of symptomatic spells

Spells may occur spontaneously or may be triggered by such factors as:

  • Physical exertion
  • Anxiety or stress
  • Changes in body position
  • Labor and delivery
  • Surgery and anesthesia

Foods high in tyramine, a substance that affects blood pressure, also can trigger a spell. Tyramine is common in foods that are fermented, aged, pickled, cured, overripe or spoiled. These foods include:

  • Some cheeses
  • Some beers and wines
  • Chocolate
  • Dried or smoked meats

Certain medications that can trigger a symptomatic spell include:

  • Monoamine oxidase inhibitors (MAOIs), such as phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan)
  • Stimulants, such as amphetamines or cocaine

When to see a doctor

The signs and symptoms of pheochromocytoma can be caused by a number of conditions. So it's important to get a prompt diagnosis.

Although high blood pressure is a primary sign of a pheochromocytoma, most people with high blood pressure don't have an adrenal tumor. Talk to your doctor if any of the following factors are relevant to you:

  • Difficulty controlling high blood pressure with current treatment plan
  • A family history of pheochromocytoma
  • A family history of a related genetic disorder: multiple endocrine neoplasia, type II (MEN II); von Hippel-Lindau disease; familial paraganglioma or neurofibromatosis 1 (NF1)

Causes

Researchers don't know what causes a pheochromocytoma. The tumor develops in specialized cells, called chromaffin cells, situated in the center of an adrenal gland. These cells release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine), that help control many body functions, such as heart rate, blood pressure and blood sugar.

The role of hormones

Adrenaline and noradrenaline are hormones that trigger your body's fight-or-flight response to a perceived threat. The hormones prompt higher blood pressure, a faster heart rate and a boost in other body systems that enable you to react quickly. A pheochromocytoma results in the irregular and excessive release of these hormones.

Related tumors

While most chromaffin cells reside in the adrenal glands, small clusters of these cells are also in the heart, head, neck, bladder, back wall of the abdomen and along the spine. Tumors in these chromaffin cells, called paragangliomas, may result in the same effects on the body.

Endocrine system

The endocrine system includes the pituitary gland, thyroid gland, parathyroid glands, adrenal glands, pancreas, ovaries (in females) and testicles (in males).

Risk factors

People with rare inherited disorders have an increased risk of developing a pheochromocytoma or paraganglioma, and tumors associated with these disorders are more likely to be cancerous. These genetic conditions include the following:

  • Multiple endocrine neoplasia, type II (MEN II) is a disorder resulting in tumors in more than one part of the body's hormone-producing (endocrine) system. Other tumors associated with MEN II can appear on the thyroid, parathyroid, lips, tongue and gastrointestinal tract.
  • Von Hippel-Lindau disease can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
  • Neurofibromatosis 1 (NF1) results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
  • Hereditary paraganglioma syndromes are inherited disorders that result in either pheochromocytomas or paragangliomas.
Multiple endocrine neoplasia, type IIB (MEN IIB)

Pheochromocytoma can occur in the inherited syndrome multiple endocrine neoplasia, type IIB (MEN IIB). In addition to pheochromocytoma, people with MEN IIB also have thyroid cancer and tumors of nerves in the lips, mouth, eyes and digestive tract.

Complications

High blood pressure can damage multiple organs, particularly tissues of the cardiovascular system, brain and kidneys. Untreated, high blood pressure associated with pheochromocytomas can result in a number of critical conditions, including:

  • Heart disease
  • Stroke
  • Kidney failure
  • Acute respiratory distress
  • Damage to the nerves of the eye

Cancerous (malignant) tumors

Rarely, a pheochromocytoma is cancerous (malignant), and the cancerous cells spread to other parts of the body (metastasize). Cancerous cells from a pheochromocytoma or paraganglioma most often migrate to the lymph system, bones, liver or lungs.

Diagnosis

Your doctor might order a number of tests.

Laboratory tests

Your doctor will likely order the following tests that measure levels of adrenaline, noradrenaline or byproducts of those hormones in your body:

  • 24-hour urine test. You will be asked to collect a urine sample every time you urinate during a 24-hour period. Ask for written instructions about how to store, label and return the samples.
  • Blood test. You will have blood drawn for laboratory work. Talk to your doctor about special preparations, such as fasting or skipping a medication. Don't skip a dose of medication without instructions from your doctor.

Imaging tests

If the results of laboratory tests indicate the possibility of a pheochromocytoma or paraganglioma, your doctor will order one or more imaging tests to locate a possible tumor. These tests may include:

  • CT scan, a specialized X-ray technology
  • MRI, which uses radio waves and a magnetic field to produce detailed images
  • M-iodobenzylguanidine (MIBG) imaging, a scanning technology that can detect tiny amounts of an injected radioactive compound taken up by certain tumors
  • Positron emission tomography (PET), a scanning technology that can also detect radioactive compounds taken up by a tumor

Incidental discovery

A tumor in an adrenal gland might be found during imaging studies conducted for other reasons. In such cases, your doctor would order additional tests to determine the nature of the tumor.

Genetic testing

Your doctor might recommend genetic tests to determine if a pheochromocytoma is related to an inherited disorder. Information about possible genetic factors can be important for a number of reasons:

  • Because some inherited disorders can cause multiple conditions, test results may indicate the need to screen for other medical problems.
  • Because some disorders are more likely to be recurrent or cancerous (malignant), your test results may affect treatment decisions or long-term plans to monitor your health.
  • Results from your tests may indicate that other family members should be screened for pheochromocytoma or related conditions.

Ask your doctor about genetic counseling services that can help you understand the benefits and implications of genetic testing.

Treatment

The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before you undergo surgery, your doctor will prescribe specific blood pressure medications that block the actions of the high-adrenaline hormones to lower the risk of developing dangerously high blood pressure during surgery.

Pre-operative medications

You will likely take two drugs for seven to 10 days that help lower blood pressure before surgery. These drugs will either replace or be added to other blood pressure drugs you take.

  • Alpha blockers keep smaller arteries and veins open and relaxed, improving blood flow and decreasing blood pressure. Alpha blockers include phenoxybenzamine (Dibenzyline), doxazosin (Cardura), and prazosin (Minipress). Side effects might include irregular heartbeat, dizziness, fatigue, vision problems, sexual dysfunction in men and swelling in your limbs.
  • Beta blockers cause your heart to beat more slowly and with less force. They also help keep blood vessels open and relaxed. In preparing for surgery a beta blocker is added several days after starting the alpha blocker.

    Beta blockers include atenolol (Tenormin), metoprolol (Lopressor, Toprol-XL) and propranolol (Inderal, Innopran XL). Possible side effects include fatigue, upset stomach, headache, dizziness, constipation, diarrhea, irregular heartbeat, difficulty breathing and swelling in the limbs.

  • High-salt diet Alpha and beta blockers widen (dilate) the blood vessels, causing the amount of fluid within the blood vessels to be low. This can cause dangerous drops in blood pressure with standing. A high-salt diet will draw more fluid inside the blood vessels, preventing the development of low blood pressure during and after surgery.

Surgery

In most cases, the entire adrenal gland with a pheochromocytoma is removed with laparoscopic, or minimally invasive, surgery. Your surgeon will make a few small openings through which he or she inserts wandlike devices equipped with video cameras and small tools.

The remaining healthy adrenal gland carries out the functions normally performed by two, and blood pressure usually returns to normal.

In some unusual situations, such as when the other adrenal gland has been removed, surgery might remove only the tumor, sparing some healthy tissue.

If a tumor is cancerous (malignant), the effectiveness of surgery might rely on removing the tumor and all cancerous tissue. However, even if all of the cancerous tissue isn't removed, surgery might limit hormone production and provide some control of blood pressure.

Cancer treatments

As cancer is rare among cases of pheochromocytoma, the research about the best treatments is relatively limited. Treatments for malignant tumors and metastasized cancer related to a pheochromocytoma might include:

  • Radionuclide treatment. This radiation therapy combines MIBG, a compound that attaches to adrenal tumors, with a type of radioactive iodine. The treatment goal is to deliver radiation therapy to a specific site and kill cancerous cells.
  • Chemotherapy. Chemotherapy is the use of powerful drugs that kill fast-growing cancer cells.
  • Targeted cancer therapies. These medications inhibit the function of naturally occurring molecules that promote the growth and spread of cancerous cells.

Preparing for an appointment

You're likely to start by seeing your primary care provider. Then you might be referred to a doctor who specializes in hormonal disorders (endocrinologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting before having a specific test. Make a list of:

  • Your symptoms, including any changes from normal, when they began and how long they last
  • Key personal information, including major stresses, recent life changes and family medical history
  • All medications, vitamins and other supplements you take, including doses
  • Questions to ask your doctor

Take a family member or friend along, if possible, to help you remember the information you get.

For a pheochromocytoma, questions to ask your doctor include:

  • What's likely causing my symptoms?
  • Other than the most likely cause, what are other possible causes for my symptoms?
  • What tests do I need?
  • Is my condition likely temporary or chronic?
  • What's the best course of action?
  • What are the alternatives to the primary approach you're suggesting?
  • I have these other health conditions. How can I best manage them together?
  • Are there restrictions I need to follow?
  • Should I see a specialist?
  • Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

  • Have your symptoms been continuous or intermittent?
  • Does anything seem to improve your symptoms?
  • What, if anything, appears to bring on or worsen your symptoms?
  • Have you been diagnosed with other medical conditions? If so, what treatment are you getting?

Last updated: April 21st, 2017

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