Find out more about the symptoms, causes, and treatment of this rare adrenal gland tumor.
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. 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.
Most pheochromocytomas are discovered in people between the ages of 20 and 50. But the tumor can develop at any age. Surgery to remove a pheochromocytoma usually returns blood pressure to normal.
Signs and symptoms of pheochromocytomas often include:
Less common signs or symptoms may include:
The symptoms listed above may be constant, or they may occur, or get stronger, occasionally. 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 doctor if any of the following factors are applicable 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), that help control many body functions, such as heart rate, blood pressure and blood sugar.
Adrenaline and noradrenaline trigger your body's fight-or-flight response to a perceived threat. The hormones cause your blood pressure to increase and your 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 causes them to be released when you're not in a threatening situation.
While most of the chromaffin cells are located 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. Chromaffin cell tumors, called paragangliomas, may result in the same effects on the body.
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. These genetic conditions include:
High blood pressure can damage multiple organs, particularly tissues of the cardiovascular system, brain and kidneys. This damage can cause a number of critical conditions, including:
Rarely, a pheochromocytoma is cancerous (malignant), 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.
Your doctor might order several tests.
The following tests measure levels of adrenaline, noradrenaline or byproducts of those hormones in your body:
For both types of tests, talk with your doctor about special preparations, such as fasting or skipping a medication. Don't skip a medication dose without instructions from your doctor.
If the lab test results suggest the presence of a pheochromocytoma or paraganglioma, your doctor will likely order one or more imaging tests to locate a possible tumor. These tests may include:
A tumor in an adrenal gland might be found during imaging studies done for other reasons. In such cases, your doctor will generally order additional tests to determine the nature of the tumor.
Your doctor 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:
Ask your doctor about genetic counseling services that can help you understand the benefits and implications of genetic testing.
The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your doctor will likely 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.
You'll 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.
Beta blockers. These drugs cause your heart to beat more slowly and with less force. They also help keep blood vessels open and relaxed. In your surgery preparation, your doctor will likely prescribe a beta blocker 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.
In most cases, your surgeon removes the entire adrenal gland with a pheochromocytoma with 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. Blood pressure usually returns to normal.
In some cases, such as when the other adrenal gland has been removed, your doctor might remove only the tumor, sparing some healthy tissue.
If a tumor is cancerous, the tumor and other cancerous tissue will be removed. However, even if all of the cancerous tissue isn't removed, surgery might limit hormone production and provide some blood pressure control.
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, include:
You're likely to start by seeing your primary care doctor. 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.
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:
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:
Don't hesitate to ask other questions.
Your doctor is likely to ask you questions, including:
December 24th, 2020