Find out more about the symptoms, causes and treatment of this rare adrenal gland tumor that is usually noncancerous.
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.
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.
Signs and symptoms of pheochromocytomas often include:
Less common signs or symptoms may include:
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:
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:
Certain medications that can make symptoms worse include:
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:
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.
The endocrine system includes the pituitary gland, thyroid gland, parathyroid glands, adrenal glands, pancreas, ovaries and testicles.
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:
People with MEN 2B have tumors of nerves in the lips, mouth, eyes and digestive tract. They also may have a tumor on the adrenal gland, called pheochromocytoma, and medullary thyroid cancer.
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:
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.
To diagnose pheochromocytoma, your health care provider will likely order several tests.
These tests measure levels of adrenaline, noradrenaline or substances that are a byproduct of those hormones in your body:
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.
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:
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.
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:
Genetic counseling can help you understand the results of your genetic testing.
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.
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.
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.
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.
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:
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.
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:
Questions to ask may include:
Don't hesitate to ask other questions during your appointment.
Your health care provider is likely to ask you questions, including: