Pheochromocytomas are tumors that arise in the adrenal gland and produce epinephrine (adrenaline) and/or norepinephrine. These hormones normally regulate the body's response to stress, and when released into the blood, they cause increases in heart rate and blood pressure, headaches, nausea, abdominal pain and palpitations.
Symptoms can occur more frequently as tumors become larger and can be brought on by stressful situations or by manipulation of the tumor during examination. Several genetic mutations can cause these tumors to run in families; these are explained further in the section on familial neuroendocrine syndromes.
The diagnosis of pheochromocytoma often includes blood or urine tests to check for elevations of hormones and imaging studies, such as computed tomography (CAT) scan or magnetic resonance imaging (MRI) to evaluate the adrenal glands. Pheochromocytomas should never be tested through a biopsy, because this can release large amounts of hormone, which can lead to a severe increase in blood pressure, causing a stroke.
The primary treatment for pheochromocytoma is surgical removal of the tumor, and usually the adrenal gland from which it arises. Prior to surgery, patients must take medication to prevent hypertensive crisis during surgery. Several medications are used to block the effects of epinephrine and norepinephrine overproduced by the tumor that cause these symptoms, such as including phenoxybenzamine (dibenzyline), calcium-channel blockers and beta-blockers.
If the tumor releases large amounts of hormone during tumor removal, these medications will help to prevent ill effects. Patients have the choice of either minimally invasive or open surgery.
Following surgery, patients are admitted to the hospital for blood pressure monitoring, and any preoperative medications are discontinued. Cure rates of most pheochromocytomas are excellent after surgery because most of these tumors are benign.
Since pheochromocytomas are commonly found in familial neuroendocrine syndromes, our physicians may recommend genetic testing for mutations in the adrenal cells as part of the patient's postoperative testing and follow-up care.