August 3, 2015
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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 managed with special medications both prior to and during surgery and is carefully monitored throughout 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, first attempts at blood pressure control utilize agents of a specific class of drugs known as alpha blockers. These agents are used prior to use of beta blockers to balance and best control the blood pressure response to anesthesia.


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

What is the prognosis with a pheochromocytoma?

Pheochromocytoma is benign in most cases, and if blood pressure-related surgical complications can be avoided, the likelihood of cure is excellent. 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.

In the low percent of these already rare tumors in which malignant behavior is evident, survival may still be quite prolonged, as the pace of the disease may still be slow. Participation in clinical trials of new therapies is strongly encouraged in the unfortunate case of metastatic pheochromocytoma. Should a pheochromocytoma be diagnosed during pregnancy, the mortality (death risk) is increased for both the mother and the fetus. Referral as soon as possible to a major center with experience with this circumstance is recommended.

REFERENCES:

eMedicine.com. Pheochromoctyoma.
<http://emedicine.medscape.com/article/124059-overview>

WebMD.com. Pheochromocytoma Treatment - General Information.
<http://www.webmd.com/cancer/tc/pheochromocytoma-treatment-health-professional-information-nci-pdq-general-information>

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


Medically Reviewed by a Doctor on 3/27/2015

Source: MedicineNet.com
http://www.medicinenet.com/pheochromocytoma/article.htm

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