Pheochromocytoma (cont.)

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How is a pheochromocytoma treated?

Surgery is the definitive treatment. Until the tumor is removed, control of blood pressure is a top priority. Controlling blood pressure before and during surgery is the trickiest part of care. There is a chance of developing an acute hypertensive crisis (a potentially dangerous, sudden, and severe rise in blood pressure) after anesthesia is given during the surgery. The blood pressure is therefore closely monitored through the procedure. Consultation with an endocrinologist is recommended to help design treatment for individual patients.

Because of the types of hormones involved in a pheochromocytoma, blood pressure control utilizes agents of a specific class of drugs known as alpha blockers. There are also certain drugs (known as tyrosine kinase inhibitors) available which inhibit the formation of hormones by the tumor. These medications can be used if needed preoperatively.

In the rare instances of pheochromocytomas that are malignant, chemotherapy or radiation therapy may be required after surgery. Trials of very specific or "targeted" new drugs have shown some promise in the treatment of pheochromocytoma. As yet, drug therapy for this disease cannot offer a possible cure, but may benefit the patient by reducing symptoms and sometimes prolonging life.

What is the prognosis with a pheochromocytoma?

In general, pheochromocytomas that are confined to the adrenal gland are benign, and when removed by surgery have excellent outcomes. Patients with recurrent pheochromocytoma tumors, tumors that are metastatic or unable to be removed surgically have a fair to poor outlook. Pheochromocytoma in a pregnant patient (a rare occurrence), has a poor outcome for the mother and fetus; the mortality (death) rate is about 50% for both patients.

Both malignant and benign pheochromocytomas can recur after surgery. The statistics vary from one study to the next, but recurrence rates average around 10%. Therefore, long-term follow-up care after surgery is very important to keep outlooks fair to good with additional appropriate treatments of medicine or surgery.

Medically reviewed by Jay B. Zatzkin, MD; American Board of Internal Medicine with subspecialty in Medical Oncology
REFERENCES: Pheochromoctyoma.
<> Pheochromocytoma Treatment - General Information.

Previous contributing authors and editors Ruchi Mathur, M.D. and Barbara K. Hecht, Ph.D.

Medically Reviewed by a Doctor on 12/16/2013

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