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Pheochromocytoma


Definition:

Pheochromocytoma is a rare tumor of adrenal gland tissue. It results in the release of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure .

Alternative Names:

Chromaffin tumors; Paraganglionoma

Causes, incidence, and risk factors:

Pheochromocytoma may occur as a single tumor or as more than one growth. It usually develops in the center (medulla) of one or both adrenal glands. Rarely, this kind of tumor occurs outside the adrenal gland, usually somewhere else in the abdomen.

Very few pheochromocytomas are cancerous.

The tumors may occur at any age, but they are most common from early to mid-adulthood.

Symptoms:

Other symptoms that can occur with this disease:

Symptoms occur in discrete attacks at unpredictable intervals and usually last 15 to 20 minutes. The attacks may increase in frequency, length, and severity as the tumor grows. High blood pressure may occur only from time to time.

Signs and tests:

The doctor will perform a physical exam. You may have high blood pressure, rapid heart rate, and fever during an attack of symptoms. Your vital signs can be normal at other times.

Tests include:

Treatment:

Treatment involves removing the tumor with surgery. It is important to stabilize blood pressure and pulse with medication before surgery. You may need to stay in the hospital with close monitoring of your vital signs.

After surgery, it is necessary to continually monitor all vital signs in an intensive care unit. When the tumor cannot be surgically removed, medication is needed to manage it. This usually requires a combination of medications to control the effects of the excessive hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.

Expectations (prognosis):

Most patients who have noncancerous tumors that are removed with surgery are still alive after 5 years. The tumors come back in less than 10% of these patients. Levels of the hormones norepinephrine and epinephrine return to normal after surgery.

Complications:

High blood pressure may continue in about 1 in 4 patients after surgery. However, standard treatments can usually control high blood pressure. In about 1 in 10 people, the tumor may return. Patients who have been successfully treated for pheochromocytoma should have testing from time to time to make sure the tumor hasn't returned. Close family members may also benefit from testing, depending on the exact type of tumor.

Calling your health care provider:

Call your health care provider if:

  • You have symptoms of pheochromocytoma
  • You had a pheochromocytoma in the past and your symptoms return
References:

National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Neuroendocrine tumors. 2012. Version 1.2012.

Young WF Jr. Adrenal medulla, catecholamines, and pheochromocytoma. In Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 235.


Review Date: 9/9/2012
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.

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