Alternate Names: Chromaffin tumors

Causes and Risks:

The tumor may be single or multiple and usually develops in the medulla (center or core) of one or both adrenal glands. Sometimes the tumors occurs outside the adrenal gland, usually within the abdomen. Less than 10% of the tumors are malignant (cancerous).

The tumors may occur at any age, but they are most common in young to mid-adult life. A common clinical feature is a paroxysm (attack of symptoms) that may be frequent but sporadic, and may increase in frequency, duration, and severity.

Prevention: There is no known prevention.

Symptoms:

• Severe headache
• Palpitations
• Rapid heart rate
• Sweating
• Flushing
• Chest pain
• Abdominal pain
• Nervousness
• Irritability
• Increased appetite
• Loss of weight

• Sleeping difficulty
• Hand tremor
• Blood pressure, high

Signs and Tests: Vital signs (temperature, pulse, rate of breathing, blood pressure) reveal high blood pressure (hypertension) that may be sustained or episodic, rapid heart rate, and elevated temperature.

Tests include:

• An adrenal biopsy that shows pheochromocytoma
• A MIBG scintiscan that shows tumor
• An MRI of abdomen that shows adrenal mass
• An abdominal CT scan that shows mass.
• Elevated urine metanephrine
• Elevated urine catecholamines
• Abnormal results of a glucose test
• Abnormal levels of catecholamines - blood

Treatment: The definitive treatment is removal of the tumor by surgery. Continuous monitoring of all vital signs is necessary in the postoperative period in an intensive care unit. Stabilization of the person’s vital signs with medication prior to surgery is important and may require hospitalization. In the case of an inoperable tumor, management with medication is necessary. Radiation therapy or chemotherapy have not been effective in destroying the tumor.

Prognosis: With surgery, the 5-year survival rate is 95%, with recurrence less than 10%. Hormone secretion of norepinephrine and epinephrine returns to normal after surgery. In malignant tumors, the 5-year survival after surgery is less than 50%.

Complications: High blood pressure may not be cured in one-fourth of the people after surgery, yet control is usually achieved in these people with standard treatments for hypertension. Recurrence of tumor may occur in 10% of the cases.

Call your health care provider if you have symptoms of pheochromocytoma.

Call your health care provider if you have had a pheochromocytoma in the past and symptoms recur.