Pheochromocytoma

Written by Amanda MacMillan
Medically Reviewed by Jabeen Begum, MD on December 05, 2024
6 min read

A pheochromocytoma (PCC) is a rare tumor that usually grows in your adrenal glands, above your kidneys. It’s also known as an adrenal paraganglioma or a chromaffin cell tumor.

It’s most common in adults aged 30 to 50 but can affect people of all ages. Children make up about 10% of all cases.

Your adrenal glands make hormones that control things such as your metabolism and blood pressure. A pheochromocytoma also releases hormones, at much higher levels than usual. These extra hormones cause high blood pressure, which can damage your heart, brain, lungs, and kidneys.

About 10%-15% of these tumors are cancerous and can spread to other parts of your body. But most pheochromocytoma tumors are benign, meaning they’re not cancerous.

Paraganglioma vs. pheochromocytoma

The names of these tumors are often interchanged, and both are neuroendocrine tumors. Paragangliomas are chromaffin cell tumors that develop outside the adrenal gland, and pheochromocytomas develop within it. A paraganglioma grows close to ganglia, which are bundles of nerve cells throughout your body. These tumors typically develop in the abdominal cavity or the head and neck. They grow slowly and are usually benign, but some types are more likely to become cancerous, including some that are inherited. Benign paragangliomas that release hormones can raise your blood pressure and cause heart problems.

Some people with these tumors have high blood pressure all the time. For others, it goes up and down.

That may be the only sign of a problem. But most people have at least one other symptom, including:

These symptoms can happen suddenly, like an attack, several times a day, or they can happen just a few times a month. As the tumor grows, the attacks may become stronger and may happen more often.

Some people’s attacks are set off by things such as:

Doctors don’t know why most PCC tumors form. About 30% of cases seem to run in families. These are more likely to be cancerous than ones that appear at random.

The tumors are more common in people who have disorders or conditions that are passed down from parents to children, including:

  • Multiple endocrine neoplasia, type 2
  • Von Hippel-Lindau disease
  • Neurofibromatosis 1 (NF1)
  • Hereditary paraganglioma syndrome

If you don’t get treatment, PCC can lead to life-threatening conditions, including:

Heart attack

Many people who have pheochromocytoma are never diagnosed because the symptoms are so much like those of other conditions. Some tests can tell your doctor if you have it, such as:

  • Blood or urine tests to look for high levels of hormones
  • An MRI scan, which uses powerful magnets and radio waves to make images of organs and tissues to check for a tumor
  • A CT scan, which puts together several X-rays taken from different angles to spot a tumor

If you have PCC, you might have tests to see if a problem with your genes caused it. This can tell you if you’re more likely to get another tumor and whether your family members are at higher risk.

You’ll most likely need surgery to remove the tumor. 

Before the surgery, you may need to take a blood pressure medicine to block adrenal gland hormones from raising blood pressure to dangerously high levels during surgery. These may include an alpha-blocker, beta-blocker, or calcium channel blocker, drugs that help your blood vessels relax and open. You will need to take these medicines one to two weeks before surgery and may be prescribed a low-sodium diet.

Your doctor might perform your surgery using tiny cuts instead of one large opening. This is called laparoscopic or minimally invasive surgery. You generally recover faster from those procedures than from traditional surgery.

If you have a tumor in only one adrenal gland, your doctor may remove that whole gland. The other gland will make the hormones your body needs.

If you have tumors in both glands, your surgeon may remove just the tumors and leave part of the glands.

If your tumor is cancerous, you may also have radiation, chemotherapy, or targeted therapy (using drugs to attack specific cancer cells) to help keep it from growing.

There's no way to prevent a pheochromocytoma from developing. But those at risk of developing the tumor due to certain inherited conditions or genes can seek genetic testing to screen for it. Talk to your doctor if you have a parent or sibling with paraganglioma or pheochromocytoma or one of the following conditions:

  • Multiple endocrine neoplasia, type 2 (MEN2)
  • Von Hippel-Lindau disease
  • Neurofibromatosis 1 (NF1)
  • Carney-Stratakis dyad
  • Carney triad

If your doctor removes both of your adrenal glands, your body won’t be able to make some necessary hormones. You can take medication to help replace those hormones.

Whatever your treatment, you’ll need regular checkups with your doctor to ensure that PCC doesn’t come back. They can also help you manage any long-term side effects of treatment.

According to estimates, about 95% of people diagnosed with a cancerous form of pheochromocytoma that hasn’t spread to other parts of their body live at least five more years. If the tumors have spread or come back after treatment, about 34%-60% of people live at least five years after diagnosis.

Pheochromocytoma is a rare type of tumor that develops in the adrenal gland. Usually benign, the best treatment for pheochromocytomas is often surgery. But treatment depends on several factors such as the tumor size, whether it is cancerous, and if it has spread to other parts of the body (metastasized). Symptoms of pheochromocytoma may also be managed by medications. Early detection is important for successfully managing and treating pheochromocytoma.

How to manage the symptoms of pheochromocytoma?

Pheochromocytoma symptoms such as high blood pressure, headaches, palpitations, and too much sweating may come and go. Medications such as alpha-blockers, which regulate your blood pressure, and beta-blockers, which steady your heart rate, may be prescribed to manage your symptoms. Some medicines can also limit the symptoms resulting from excess hormones released from the adrenal gland.

What does a pheochromocytoma attack feel like?

During a pheochromocytoma attack, you may develop a headache or irregular heartbeat and sweat heavily. You may feel as if you're having a panic attack with symptoms such as:

  • Nervous trembling
  • A paler skin tone (pallor)
  • Shortness of breath
  • Vision problems
  • Anxiety
  • Feelings of fear or doom

Is pheochromocytoma cancer?

Pheochromocytoma is a type of tumor, which means it can be benign (not cancerous) or cancerous. Most pheochromocytomas are not cancerous, but around 10%-15% may be.

Do pheochromocytoma symptoms come and go?

Yes, pheochromocytoma symptoms, including high blood pressure, can come and go. Sometimes, the symptoms appear daily, while at other times you may have a few episodes a month.

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