Pheochromocytoma: An Adrenal Gland Tumor

What is a pheochromocytoma?

A pheochromocytoma is a rare type of tumor. It grows inside the middle part of an adrenal gland. Your body has two adrenal glands. They are found on top of each kidney. Each layer of these glands makes different hormones. The middle part of the adrenal glands makes epinephrine and norepinephrine. These hormones help keep your heart rate and blood pressure normal. A pheochromocytoma causes the adrenal glands to make too much of these hormones.

What causes pheochromocytoma?

Experts don't know what causes this type of tumor. But there may be a genetic component.

Who is at risk for pheochromocytoma?

Pheochromocytomas happen equally in men and women. They often show up when you are in your 30s, 40s, or 50s.

If someone in your family has this type of tumor, you may be at a higher risk of developing it. This tumor does not seem to be affected by environment, diet, or lifestyle. If you have this tumor, you should think about genetic testing. About 1 in 4 of these types of tumors are now believed to run in families.

What are the symptoms of pheochromocytoma?

The most common sign of a pheochromocytoma is high blood pressure. It can be always high or sometimes high. Sometimes the tumor can cause high blood pressure that can be life-threatening. It's a very rare cause of high blood pressure. But it must be considered when medicine is not enough to control high blood pressure.

Other symptoms are less common. They can be brought on when you are under stress or when you change positions. Each person’s symptoms may vary. Symptoms may include:

• Very fast pulse

• Feeling that your heart is beating fast or fluttering (palpitations)

• Pounding heartbeat

• Headache

• Nausea

• Vomiting

• Clammy skin and sweating

• Shaking (tremors)

• Anxiety

• Flushing

The symptoms of a pheochromocytoma may seem like other health problems. Always see your healthcare provider for a diagnosis.

How is pheochromocytoma diagnosed?

Your healthcare provider will take your health history and give you a physical exam. You may also need tests, such as:

• Blood and urine tests. These tests measure hormone levels. Genetic tests may also be done to check if you have a genetic condition that may raise your risk..

• CT scan. This test uses X-rays and computer technology to take detailed 3-D images of your body.

• MRI. This test uses large magnets, radio waves, and a computer to also make 3-D images of organs and structures within your body.

• Radioisotope scan. This test puts radioactive substances into your body to get an image of the tumor. The radioactive substance is absorbed into tissues that make too much of the hormone epinephrine. During the scan, any area that absorbs the radioactive substance can be seen.

How is a pheochromocytoma treated?

Treatment will depend on your symptoms, age, and general health. It will also depend on how severe the condition is.

Treatment may include: 

• Surgery. This is the most common treatment. The surgeon may remove parts of the gland or one or both adrenal glands. Before surgery. you may start medicine to block the effect of the adrenal hormones.

• Medicine. If you can't have surgery, you may take medicine. But this is rarely done. The tumor is mainly treated with surgery.

Living with a pheochromocytoma

Most of these tumors are benign. This means they are not cancer, but they have the ability to spread Many will not come back after they are removed. In a small number of cases, the tumor may come back or spread to other places in the body and may become cancerous.

When should I call my healthcare provider?

Tell your healthcare provider if your symptoms come back or get worse. Also tell your provider if you have any new symptoms.

Key points about pheochromocytomas

• A pheochromocytoma is a tumor in the adrenal gland. It causes the gland to make too much of the hormones epinephrine and norepinephrine.

• This tumor often occurs when you are in your 30s, 40s, or 50s. It happens to both men and women.

• Experts don't know what causes these tumors.

• The most common sign of this tumor is high blood pressure.

• Most of these tumors are not cancer. They will not come back after they are removed.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new directions your provider gives you.

• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions, especially after office hours and on weekends and holidays.