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Pheochromocytoma is a rare and specialized type of tumor of the chromaffin cells. Chromaffin cells are primarily found in the body’s two adrenal glands, and about 90 percent of pheochromocytomas occur in the adrenal glands. Located above each kidney in back of the upper abdomen, the adrenal glands produce catecholamines, a group of hormones including norepinephrine, adrenaline and dopamine, which help regulate blood pressure and heart rate.
If too many catecholamines are released into the body, the patient will develop severe high blood pressure. Left untreated, high blood pressure can lead to heart attack, stroke or other major health problems.
Pheochromocytomas may appear singly or in groups. In about 10 percent of cases that arise in the adrenal glands, both glands are affected. Besides the adrenal glands, pheochromocytomas may occur in the chest or, very rarely, in the neck.
Pheochromocytoma is sometimes referred to as the “10 percent tumor” because of the number of factors that appear in only about 10 percent of cases, including:
- Being cancerous (90 percent of pheochromocytomas are benign).
- Occurring in children (90 percent of pheochromocytoma cases are adults).
- Occurring in both adrenal glands. This condition is called bilateral pheochromocytoma (90 percent affect only one adrenal gland).
- Beginning in areas outside the adrenal gland, such as the aorta, bladder, kidney or along the spinal cord. These are called extra-adrenal pheochromocytomas (90 percent of cases begin in the adrenal gland).
- Occurring in members of the same family (familial pheochromocytoma). Only about 10 percent of patients with pheochromocytoma have a family member who also has this condition.
- Occurring as part of multiple endocrine neoplasia syndrome (MEN). Patients with MEN often have multiple cancerous tumors in other glands in the body (such as the thyroid gland) as well as other hormonal disorders.
- Occurring after a stroke (10 percent of cases are discovered after a patient has a stroke).
- Recurring within five to 10 years of initial diagnosis (recurrent pheochromocytoma). Less than 10 percent of all pheochromocytoma cases are recurrent.
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