A pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare, usually noncancerous (benign) tumor that develops in cells in the center of an adrenal gland. You have two adrenal glands, one above each kidney. Your adrenal glands produce hormones that give instructions to virtually every organ and tissue in your body.
If you have a pheochromocytoma, an adrenal gland releases hormones that cause persistent or episodic high blood pressure. If left 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.
Signs and symptoms of pheochromocytomas often include:
Less common signs or symptoms may include:
These signs and symptoms often occur in brief spells of 15 to 20 minutes. Spells can happen several times a day or less often. Your blood pressure may be within the normal range or remain elevated between spells.
Spells may occur spontaneously or may be triggered by such factors as:
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 may include:
Certain medications that can trigger a symptomatic spell include:
The signs and symptoms of pheochromocytoma can be caused by a number of different conditions. Therefore, 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:
Researchers don't know exactly what causes the development of a pheochromocytoma. They do understand that the tumor develops in specialized cells, called chromaffin cells, located in the center of an adrenal gland. These cells produce and release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine).
Adrenaline and noradrenaline are hormones that normally function as the trigger for 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 with a burst of energy. A pheochromocytoma results in the irregular and excessive release of these hormones.
While most chromaffin cells reside in the adrenal glands, small clusters of these cells are also located 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.
Your doctor may order a number of tests.
Your doctor will likely order the following tests that measure levels of adrenaline, noradrenaline or byproducts of those hormones in your body:
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:
A tumor in an adrenal gland may be discovered incidentally during imaging studies conducted for other reasons. In such cases, your doctor would order additional tests to determine the nature of the tumor.
Your doctor may 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:
Ask your doctor about genetic counseling services that can help you understand the benefits and implications of genetic testing.
High blood pressure can damage multiple organs, particularly tissues of the cardiovascular system, brain and kidneys. If left untreated, high blood pressure associated with pheochromocytomas may result in a number of critical conditions, including:
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.
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: