Pheochromocytoma (PCC)

Pheochromocytoma

What is a pheochromocytoma? — A pheochromocytoma is a rare type of tumor. It usually happens in or near the adrenal glands. These are small organs that sit on top of each kidney (figure 1).
A pheochromocytoma makes too much of a hormone called "adrenaline." This is the "fight or flight" hormone. Having too much of this hormone can cause attacks of very high blood pressure, fast heart rate, sweating, and headaches. Some people call these attacks "spells."
Usually, pheochromocytomas happen in adults. But they sometimes happen in children and they can run in families. People with rare diseases such as multiple endocrine neoplasia (also called "MEN") and von Hippel-Lindau disease are more likely to have pheochromocytomas.
They are more likely to be cancerous in children than in adults. But a pheochromocytoma can be serious even if there is no cancer. The "fight or flight" hormone it makes can damage the heart and even cause death.
What are the symptoms of a pheochromocytoma? — Symptoms are different for each person. Adults and children can have different symptoms.
Common symptoms include:
Headaches – These can be mild or severe, and last a long or short time.
Sweating
Fast heartbeat, or feeling like the heart is beating very hard or skips beats
High blood pressure
Shaking
Looking pale
Feeling short of breath, weak, or both
Feeling fearful or anxious
Belly pain or swelling – This is more likely in children.
Some people with pheochromocytomas do not have any symptoms. They might learn that they have this after getting an imaging test, like a CT scan or MRI, for another reason. (Imaging tests create pictures of the inside of the body.) Other people find out they have it when their blood pressure gets extremely high during a procedure or surgery.
Many people with pheochromocytomas have very high blood pressure that can be dangerous. Blood pressure can go up and down if a person changes position or for other reasons.
Will I need tests? — Yes. Your doctor or nurse will talk with you and do an exam. He or she will also do lab tests to measure the hormone levels in your body. These include:
Blood tests
Urine tests
Before you have tests, tell the doctor or nurse about any medicines you take. This includes prescription and over-the-counter medicines. You might need to stop taking them before having tests. Some medicines change hormone levels, and this could make the test results wrong.
If blood or urine tests show someone has a pheochromocytoma, doctors do tests to find it in the body. These include:
CT or MRI scans of the adrenal glands
Other imaging tests, if the CT or MRI does not show a tumor in the adrenal glands.
How is a pheochromocytoma treated? — A pheochromocytoma is treated with surgery to remove it.
Before surgery, doctors give medicine to get the blood pressure and heart rate under control. This helps prevent serious problems during surgery. Medicine is given for about 2 weeks before surgery.
Pheochromocytomas are usually not cancerous. But if a pheochromocytoma is cancerous, doctors take out as much of it as possible. They can also treat cancer with radiation, chemotherapy, or other treatments.
Most people are cured with surgery. But a pheochromocytoma can also come back, even after surgery. Sometimes, pheochromocytomas that were not cancerous at first come back as cancer. After treatment, plan to get checkups as often as the doctor recommends.
What if I am pregnant? — If you are pregnant, the doctor can give medicine to help control symptoms from the pheochromocytoma. You might have surgery to remove it if you are less than 24 weeks pregnant.
When your baby has grown enough to live outside your body, the doctor can do a c-section (surgery to get the baby out). He or she can remove the tumor at the same time. If you have a pheochromocytoma, having a c-section is safer than giving birth through your vagina.
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This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 17223 Version 7.0
Release: 28.2.2 - C28.105
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