Dr. M.J. Bazos, MD. Patient Handout

About Your Diagnosis
Pheochromocytoma is a tumor of the adrenal gland or the sympathetic nervous system chain that secretes adrenaline (epinephrine) or related compounds. This causes symptoms of high blood pressure, heart palpitations, headaches, and sweats that come and go over time. Doctors do not know what causes pheochromocytomas to form. Most cases occur sporadically, but approximately 10% are part of familial endocrine tumor syndromes. Pheochromocytomas are uncommon. They account for only 0.1% to 0.01% of all patients with hypertension. Patients may complain of episodic spells of hypertension, headaches, sweats, or anxiety. Alternatively, a tumor may be noted on the adrenal glands while examining the abdomen for other reasons.
Pheochromocytomas are diagnosed by measuring catecholamines in a 24-hour urine collection. It is important that the patient be in a nonstressful environment when this test is done, and that the patient not be consuming alcohol, caffeine, amphetamines, benzodiazepines, certain antidepressants, or lithium, which may falsely elevate urinary catecholamine levels. Once excess catecholamine secretion has been documented with the urine test, a magnetic resonance imaging (MRI) of the adrenal glands with T2-weighted images will localize the tumor. Ten percent of tumors lie outside the adrenals. These patients may require whole-body imaging with special nuclear medicine tests. More than 90% of pheochromocytomas are curable by surgery because they are contained within the adrenal gland. Ten percent of these tumors, however, are malignant and are not curable with surgery. Such patients require a combination of treatments to control their disease.

Living With Your Diagnosis
Episodic headaches, anxiety, palpitations (strong heart beats), sweats, high blood pressure, heat intolerance, and dizziness when standing are common symptoms. Some individuals may have no symptoms. Uncontrolled hypertension can cause loss of vision, heart disease, kidney disease, and strokes.

More than 90% of pheochromocytomas are confined to the adrenal glands and are cured with surgery. The experience of the surgeon is critical for the success of the operation. Appropriate preoperative treatment must be instituted to ensure a safe operation. Complications from surgery include bleeding and infection, and transient low and high blood pressure reactions that can occur while the tumor is being removed. Rapid intraoperative response is required by the anesthesiologist. A catheter may be placed into a central vein to carefully monitor your cardiovascular status. Patients should be started on a blood pressure medicine called phenoxybenzamine or another similar medicine preoperatively. Phenoxybenzamine may cause low blood pressure upon standing (orthostatic hypotension). Eat a high-salt diet and drink plenty of fluids to prevent this side effect. Phenoxybenzamine can also cause dry mouth, nasal congestion, and dizziness. The dose needs to be carefully adjusted preoperatively. A second medicine called a beta blocker may be added after the phenoxybenzamine has been started. Beta blockers help prevent racing heartbeats. Other blood pressure medicines may be used as well. Malignant tumors should be surgically resected to remove as much tumor tissue as possible. Most patients will then be started on phenoxybenzamine postoperatively to control blood pressure. Usually, a combination of chemotherapy, radiation therapy, or other treatments are used to help control the spread of the disease.

The DOs
• Increase your fluid and salt intake preoperatively to prevent dizziness.
• Have your blood pressure checked both lying down and standing preoperatively.
• Rest as much as possible until your tumor has been removed to minimize extra stress to your heart.
• Inform your doctor if you have a history of pheochromocytomas, or other endocrine tumors in your family. Family members may need screening blood work or urine testing.

The DON’Ts
• Don’t take a diuretic medicine (unless specifically ordered by your doctor) before your operation. This can lead to dehydration.
• Don’t take the beta blocker medication if you are not already on phenoxybenzamine or a similar compound. Beta blockers alone could lead to severely elevated blood pressures in patients with pheochromocytomas.
• Don’t perform strenuous exercise until your pheochromocytoma has been removed.
• Don’t expect that your high blood pressure will completely normalize after the operation. Some permanent changes may have already occurred in the kidneys and blood vessels.
• Don’t forget to see your doctor on a regular basis to make sure that the tumor has not returned.

When to Call Your Doctor
• You have a change in your vision, a severe headache, weakness on one side of the body, chest pains, or increasing palpitations.
• You have ankle swelling or shortness of breath.
• You have weakness or dizziness when standing.
• Your symptoms return postoperatively.