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Orthopedics

Pheochromocytoma in Children

What is a pheochromocytoma in children?

Pheochromocytoma is a tumor that develops in the medulla (center) of the adrenal glands. The tumor makes hormones called epinephrine and norepinephrine. This leads to an excess of the catecholamine hormones in the body. These hormones help manage heart rate, blood pressure, and other tasks. Too much of these hormones in the body causes problems. Pheochromocytoma is rare. It occurs most often in adults between ages 20 and 50. But about 10% of cases are in children. A child may have more than 1 tumor.

What causes a pheochromocytoma in a child?

The tumor often occurs by chance (sporadic). But it is also often linked to genetic mutations that run in families. About one-quarter of the cases are part of a hereditary disease, such as:

  • Neurofibromatosis

  • Von Hippel-Lindau disease

  • Multiple endocrine neoplasia (MEN) syndromes

  • Succinate Dehydrogenase mutations (SDHx)

Which children are at risk for pheochromocytoma?

A child is at higher risk for pheochromocytoma if they have any of these:

  • Neurofibromatosis

  • Von Hippel-Lindau disease

  • Multiple endocrine neoplasia (MEN) syndromes

  • Succinate Dehydrogenase mutations (SDHx)

What are the symptoms of pheochromocytoma in a child?

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

  • Headache

  • Fast heart rate

  • Sweating

  • Pale skin (pallor)

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

  • Upset stomach (nausea)

  • Vomiting

  • Belly (abdominal) pain or bloating 

  • Back pain

  • Moist skin

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

How is pheochromocytoma diagnosed in a child?

The healthcare provider will ask about your child’s symptoms and health history. They may also ask about your family’s health history. Your child will have 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 large magnets and a computer to make detailed images of tissues in the body without the use of X-rays.

  • CT scan of the adrenal glands. 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 is specialized blood testing that looks for genetic diseases as a possible cause of the tumor.

How is pheochromocytoma treated in a child?

Treatment is done by removing the tumor via 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 in a child?

Complications can include heart damage or stroke caused by high blood pressure, or high blood glucose (diabetes mellitus).

How can I help my child live with pheochromocytoma?

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 they have episodes that include a headache, fast heart rate, and sweating. If your child has high blood pressure or other symptoms of pheochromocytoma, talk with their healthcare provider.

Key points about pheochromocytoma in children

  • Pheochromocytoma is a tumor of the adrenal glands. The tumor makes hormones called epinephrine and norepinephrine. This leads to an excess of the catecholamine hormones in the body. A child may have more than one tumor.

  • These hormones help manage heart rate, blood pressure, and 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, pallor, and sweating.

  • Treatment is done by removing the tumor 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, or high blood glucose.

  • 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 healthcare provider:

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

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

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

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

  • Ask if your child’s 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 your child does not take the medicine or have the test or procedure.

  • 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.

Online Medical Reviewer: Raymond Kent Turley BSN MSN RN
Online Medical Reviewer: Ricardo Rafael Correa Marquez MD
Online Medical Reviewer: Sabrina Felson MD
Date Last Reviewed: 2/1/2023
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