Pheochromocytoma

What is a pheochromocytoma?

A pheochromocytoma is a tumor that secretes epinephrine and norepinephrine hormones. These hormones are responsible for regulating heart rate and blood pressure, among other functions. Pheochromocytomas occur most frequently in adults between ages 20 and 50. Ten percent of individuals affected are children who usually develop symptoms between ages 6 and 14.

What causes pheochromocytomas?

The causes of pheochromocytoma are multifactorial, meaning "many factors" contribute to the cause of the condition. In most cases, both genetic and environmental factors play a role. About 25 percent of cases are part of a hereditary syndrome. Pheochromocytoma can occur alone or in combination with other disorders. The following are the most common disorders associated with pheochromocytoma:

• Neurofibromatosis

• Von Hippel-Lindau disease

• Multiple endocrine neoplasia (MEN) syndromes

What are the symptoms of pheochromocytoma?

The most common symptom of pheochromocytoma is high blood pressure, which is sometimes extreme. Each person may experience symptoms differently. Other symptoms may include:

• Rapid pulse

• Heart palpitations

• Headache

• Dizziness

• In children, poor weight gain despite good appetite

• Nausea

• Vomiting

• Abdominal pain or distension 

• Pale skin

• Clammy skin

• Sweating

The symptoms of pheochromocytoma may resemble other problems or medical conditions. Always consult a doctor for a diagnosis.

How is pheochromocytoma diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for pheochromocytoma may include:

• Blood and urine tests.  These tests measure hormone levels.

• Computed tomography scan (also called a CT or CAT scan). A diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays. Your doctor may also consider an MRI as the initial diagnostic test. 

• Radioisotope scan. This scan uses radioactive substances introduced into the body to create an image of the functioning adrenal gland.

• Genetic testing. Any child having a pheochromocytoma should be considered for genetic screening as a possible cause. 

Treatment for pheochromocytoma

Specific treatment for pheochromocytoma will be determined by a doctor based on:

• Age, overall health, and medical history

• The extent of the disease

• The type of disease

• Your tolerance for specific medications, procedures, or therapies

• Expectations for the course of the disease

• Your opinion or preference

Treatment for pheochromocytoma usually includes removing the tumor. Before removing the tumor, however, your doctor may prescribe medications to control high blood pressure. In children, there may be multiple tumors. An extensive evaluation to locate them will be required before surgery.

Continuous medical follow-up may be required to monitor the development of future tumors.

Family members may also want to consider testing to rule out the presence of the tumors, as genetics does appear to play a role in some cases.