Pheochromocytoma

Chromaffin tumors; Paraganglionoma

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. When it does, it is 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.

• Abdominal pain
• Chest pain
• Irritability
• Nervousness
• Pallor
• Palpitations
• Rapid heart rate
• Severe headache
• Sweating
• Weight loss

Other symptoms that can occur with this disease:

• Hand tremor
• High blood pressure
• Sleeping difficulty

Symptoms occur during attacks, which happen when the tumor releases hormones. The attacks usually last 15 to 20 minutes. The attacks may increase in frequency, length, and severity as the tumor grows. High blood pressure may occur from time to time.

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:

• Abdominal CT scan
• Adrenal biopsy
• Catecholamines blood test (serum catecholamines)
• Glucose test
• Metanephrine blood test (serum metanephrine)
• MIBG scintiscan
• MRI of abdomen
• Urine catecholamines
• Urine metanephrines

Treatment involves removing the tumor with surgery. It is important to stabilize your blood pressure and pulse with certain medications before surgery. You may need to stay in the hospital and have your vital signs closely monitored around the time of surgery. After surgery, your vital signs will be continuously monitored in an intensive care unit.

When the tumor cannot be surgically removed, you will need to take medication to manage it. A combination of medications is usually needed to control the effects of the excessive hormones. Radiation therapy and chemotherapy have not been effective in curing this kind of tumor.

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.

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 has not returned. Close family members may also benefit from testing, because some cases are inherited.

Call your health care provider if:

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