Health Encyclopedia

Pheochromocytoma in Children

What is a pheochromocytoma?

Pheochromocytoma is a tumor of the adrenal glands. The tumor makes hormones called epinephrine and norepinephrine. This leads to an excess of the hormones in the body. These hormones help manage heart rate and blood pressure, and they have other tasks. Too much of these hormones in the body causes problems. Pheochromocytoma is rare and occurs most often in adults from ages 20 and 50. But about 10% of cases are in children ages 6 to 14. A child may have more than one tumor.

What causes a pheochromocytoma?

The tumor can be caused by both genes and environmental factors. About a quarter of the cases are part of a hereditary disease such as:

  • Neurofibromatosis
  • Von Hippel-Lindau disease
  • Multiple endocrine neoplasia (MEN) syndromes

Who is at risk for pheochromocytoma?

A child is at higher risk for pheochromocytoma if he or she has any of these:

  • Neurofibromatosis
  • Von Hippel-Lindau disease
  • Multiple endocrine neoplasia (MEN) syndromes

What are the symptoms of pheochromocytoma?

The most common symptom is high blood pressure, which can be very high. The high blood pressure can cause:

  • Headache
  • Fast heart rate
  • Sweating

Other symptoms can occur a bit differently in each child. They may include:                                                               

  • Fast pulse
  • Heart palpitations
  • Dizziness
  • Poor weight gain despite a good appetite
  • Nausea
  • Vomiting
  • Belly (abdominal) pain or bloating 
  • Pale skin
  • Moist skin

The symptoms of pheochromocytoma can be like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is pheochromocytoma diagnosed?

The healthcare provider will ask about your child’s symptoms and health history. He or she may also ask about your family’s health history. He or she will give your child a physical exam. Your child may also have tests, such as:

  • Blood tests and urine tests. These tests measure hormone levels.
  • MRI. This test uses a large magnets and a computer to make detailed images of tissues in the body.
  • CT scan. This test uses a series of X-rays and a computer to make detailed images of the body. A CT scan can show bones, muscles, fat, and organs. CT scans are more detailed than regular X-rays.
  • Radioisotope scan. This test uses radioactive substances to create an image of the adrenal gland.
  • Genetic testing. This testing looks for genetic diseases as a possible cause of the tumor. 

How is pheochromocytoma treated?

Treatment is done by removing the tumor or tumors with surgery. Before surgery, your child’s healthcare provider may prescribe medicine to lower the high blood pressure. Most people are cured with surgery. But in some people, the tumor can grow back.

What are possible complications of pheochromocytoma?

Complications can include heart damage or stroke caused by high blood pressure.

How is pheochromocytoma managed?

Ongoing medical care may be needed to check for growth of a new tumor. Family members may also want to consider genetic testing to see who else may be at risk for pheochromocytoma.

When should I call my child's healthcare provider?

Get medical care for your child right away if he or she has episodes that include a headache, fast heart rate, and sweating. If your child has high blood pressure or other symptoms of pheochromocytoma, talk with your child's healthcare provider.        

Key points about pheochromocytoma

  • Pheochromocytoma is a tumor of the adrenal glands. The tumor makes hormones called epinephrine and norepinephrine. This leads to an excess of the hormones in the body. A child may have more than one tumor.
  • These hormones help manage heart rate and blood pressure, and have other tasks.
  • The most common symptom is high blood pressure, which can be very high. The high blood pressure can cause headache, fast heart rate, and sweating.
  • Treatment is done by removing the tumor or tumors with surgery. Most people are cured with surgery. But in some people, the tumor can grow back.
  • Complications can include heart damage or stroke caused by high blood pressure.
  • Family members may also want to consider genetic testing to see who else may be at risk for pheochromocytoma.

Next steps

Tips to help you get the most from a visit to your child’s health care provider:
  • Before your visit, write down questions you want answered.
  • At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.


Medical Reviewers:

  • MMI board-certified, academically affiliated clinician
  • Turley, Ray, BSN, MSN