Pheochromocytoma

A pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare, usually noncancerous (benign) tumor that develops in an adrenal gland. You have two adrenal glands — one located at the top of each kidney. The adrenal glands are part of the body's hormone-producing (endocrine) system. The endocrine system also includes the pituitary gland, thyroid gland, parathyroid glands, pancreas, ovaries (in females) and testicles (in males).

Usually, a pheochromocytoma develops in only one adrenal gland. But tumors can develop in both.

If you have a pheochromocytoma, the tumor releases hormones that may cause high blood pressure, headache, sweating and symptoms of a panic attack. If a pheochromocytoma isn't treated, severe or life-threatening damage to other body systems can result.

Surgery to remove a pheochromocytoma usually returns blood pressure to normal.

Symptoms

Signs and symptoms of pheochromocytomas often include:

• High blood pressure
• Headache
• Heavy sweating
• Rapid heartbeat
• Tremors
• Pallor
• Shortness of breath
• Panic attack-type symptoms

Less common signs or symptoms may include:

• Anxiety or sense of doom
• Blurry vision
• Constipation
• Weight loss

Symptomatic spells

The symptoms of pheochromocytoma may be constant, or they may occur, or get stronger, occasionally. These spells can happen because of activities or conditions, foods high in a substance that affects blood pressure (tyramine), and medications.

Certain activities or conditions can make symptoms worse, such as:

• Physical hard work
• Anxiety or stress
• Changes in body position, such as going from sitting or lying down to standing
• Labor and delivery
• Surgery and a drug that causes you to be in a sleep-like state during surgery (anesthesia)

Foods high in tyramine, a substance that affects blood pressure, also can make symptoms worse. 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 make symptoms worse 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

Although high blood pressure is a primary sign of a pheochromocytoma, most people who have high blood pressure don't have an adrenal tumor. Talk to your health care provider if any of these factors apply to you:

• Difficulty controlling high blood pressure with current treatment
• Spells of worsening high blood pressure
• A family history of pheochromocytoma
• A family history of a related genetic disorder: multiple endocrine neoplasia, type 2 (MEN 2), von Hippel-Lindau disease, hereditary paraganglioma syndromes or neurofibromatosis 1

Causes

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

Adrenaline and noradrenaline trigger the body's fight-or-flight response to a perceived threat. The hormones cause blood pressure to increase and the heart to beat faster. They prepare other body systems that enable you to react quickly. A pheochromocytoma causes more of these hormones to be released, and it causes them to be released when you're not in a threatening situation.

Most of the chromaffin cells are in the adrenal glands. But small clusters of these cells are also in the heart, head, neck, bladder, back wall of the stomach (abdomen) and along the spine. Chromaffin cell tumors located outside of the adrenal glands are called paragangliomas. They may cause the same effects on the body as a pheochromocytoma.

Risk factors

Most pheochromocytomas are discovered in people between the ages of 20 and 50. But the tumor can develop at any age.

People who have certain rare inherited disorders have an increased risk of pheochromocytoma or paraganglioma. Tumors associated with these disorders are more likely to be cancerous. Tumors are also more likely to occur in both adrenal glands. These genetic conditions include:

• Multiple endocrine neoplasia, type 2 (MEN 2) is a disorder that results in tumors in more than one part of the endocrine system. There are two types of MEN, type 2A and type 2B, that both involve pheochromocytomas. Other tumors associated with this condition can appear on other parts of the body, including the thyroid, parathyroid, lips, tongue and digestive system.
• Von Hippel-Lindau disease can cause tumors at many sites, including the central nervous system, endocrine system, pancreas and kidneys.
• Neurofibromatosis 1 causes many 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.

Complications

High blood pressure can damage organs, particularly tissues of the heart and blood vessel (cardiovascular) system, brain and kidneys. This damage can cause several critical conditions, including:

• Heart disease
• Stroke
• Kidney failure
• Problems with the nerves of the eye

Cancerous tumors

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

Diagnosis

To diagnose pheochromocytoma, your health care provider will likely order several tests.

Lab tests

These tests measure levels of adrenaline, noradrenaline or substances that are a byproduct of those hormones in your body:

• 24-hour urine test. In this test, you 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. A specialist will draw blood to be tested in the lab.

For both types of tests, talk with your health care provider about special preparations. For example, you may be asked not to eat for a period of time before the test (fast) or skip a medication. Don't skip a medication dose without instructions from your provider.

Imaging tests

If the lab test results suggest the presence of a pheochromocytoma or paraganglioma, your health care provider will likely order one or more imaging tests to find 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 pheochromocytomas or paragangliomas
• Positron emission tomography (PET), a scanning technology that also can detect radioactive compounds taken up by a tumor

A tumor in an adrenal gland might be found during imaging studies done for other reasons. If that happens, your health care provider will generally order additional tests to find out more about the tumor.

Genetic testing

Your health care provider might recommend genetic tests to determine whether a pheochromocytoma is related to an inherited disorder. Information about possible genetic factors can be important for many reasons:

• Some inherited disorders can cause multiple conditions, so test results may suggest the need to screen for other medical problems.
• Some disorders are more likely to occur again or be cancerous, so your test results may affect treatment decisions or long-term plans to monitor your health.
• Results from tests may suggest that other family members should be screened for pheochromocytoma or related conditions.

Genetic counseling can help you understand the results of your genetic testing.

Treatment

The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your health care provider will likely prescribe specific blood pressure medications. These medications block high-adrenaline hormones to lower the risk of developing dangerously high blood pressure during surgery.

Preparations before surgery

You'll likely take medications for 7 to 10 days that help lower blood pressure before surgery. These drugs will either replace or be added to other blood pressure medications you take. You may also be encouraged to eat a high-salt diet.

Medications, such as alpha blockers, beta blockers and calcium channel blockers, keep smaller veins and arteries open and relaxed. This improves blood flow and decreases blood pressure. Some of these medications may also cause your heart to beat more slowly and with less force. This can further decrease blood pressure.

Because these medications widen the blood vessels, they cause 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 low blood pressure during and after surgery.

Surgery

Usually, the procedure for an adrenal tumor is performed using minimally invasive surgery (laparoscopic surgery). Your surgeon will make a few small cuts (incisions) in your stomach (abdomen) to insert wandlike devices equipped with video cameras and small tools to perform the surgery.

In most cases, your surgeon removes the entire adrenal gland with a pheochromocytoma. However, your surgeon might remove only the tumor, leaving some healthy adrenal gland tissue. This may be done when the other adrenal gland has also been removed or when there are tumors in both adrenal glands.

If a tumor is cancerous, the tumor and other cancerous tissue will be removed. Even if all the cancerous tissue isn't removed, surgery and medical therapy might reduce hormone production and provide some blood pressure control.

After surgery

The remaining healthy adrenal gland can carry out the functions normally performed by two glands. Blood pressure usually returns to normal. You'll need regular medical appointments with your health care provider for life to monitor your health, identify complications or see if the tumor has returned.

Cancer treatments

Very few pheochromocytomas are cancerous. As such, research about the best treatments is limited. Treatments for cancerous tumors and cancer that has spread in the body, related to a pheochromocytoma, may include:

• Targeted therapies using a drug or compound that seeks out cancer cells, combined with a radioactive substance that kills them. Drug treatments that focus on specific abnormalities within cancer cells that allow them to survive may also be an option.
• Chemotherapy using powerful drugs that kill fast-growing cancer cells.
• Radiation therapy that may relieve symptoms of tumors that have spread to the bone and are causing pain.

Preparing for an appointment

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

Here's some information to help you get ready for your appointment. You may want to take a family member or friend along, if possible, to help you remember the information.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as fasting or making medication changes before having a specific test.

Before your appointment make a list of:

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

Questions to ask may 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 ongoing?
• What treatment do you recommend?
• What are the alternatives to the primary approach you're suggesting?
• I have 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 during your appointment.

What to expect from your doctor

Your health care provider is likely to ask you questions, including:

• Have your symptoms been continuous, or do they come and go?
• Does anything seem to improve your symptoms?
• What, if anything, appears to trigger or worsen your symptoms?
• Have you been diagnosed with other medical conditions? If so, what treatment are you getting?
• Do you have a family history of adrenal or other endocrine tumors?

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