Overview
A paraganglioma (also known as an extra-adrenal pheochromocytoma) is a rare neuroendocrine tumor (NET) that forms near your carotid artery (the major blood vessels in your neck), along nerve pathways in your head and neck and in other parts of your body. The tumor is made of a certain type of cell called chromaffin cells, which produce and release certain hormones known as catecholamines.
Your adrenal glands, the two glands that are on top of each kidney, make several hormones. Among these are hormones called catecholamines that help control the following important bodily functions:
- Heart rate.
- Blood pressure.
- Blood sugar (blood glucose).
- The way your body responds to stress.
The primary catecholamines include:
- Dopamine.
- Epinephrine (adrenaline).
- Norepinephrine (noradrenaline).
Even though paragangliomas don’t form in your adrenal glands, they’re made of tissue that’s found in your adrenal glands. Paragangliomas may release extra catecholamines into your blood, causing certain signs and symptoms.
What is the difference between paraganglioma and pheochromocytoma?
Paraganglioma and pheochromocytoma are both rare tumors that form from the same type of cells known as chromaffin cells. The difference is where they form in your body.
Pheochromocytomas form in the center of your adrenal gland (adrenal medulla), and paragangliomas form outside your adrenal gland, usually along the arteries or nerves in your neck. Paragangliomas are also called extra-adrenal pheochromocytomas.
Is paraganglioma cancer?
Paragangliomas can be benign (not cancer) or malignant (cancer). Approximately 20% of paragangliomas are malignant.
It can be very challenging for healthcare providers to tell if a paraganglioma is cancerous or not — even after they’ve looked at the tumor tissue under a microscope after it’s been removed. Because of this, a paraganglioma is often considered cancer if it has:
- Spread to nearby tissues (regional spread of paraganglioma).
- Spread to distant areas like your lungs or bone (metastasized).
- Come back after initial treatment (recurred).
There’s no standard staging system for paraganglioma if it’s cancerous. Instead, it’s described as the following:
- Localized paraganglioma: The tumor is in one area only.
- Regional paraganglioma: Cancer has spread to lymph nodes or other tissues near its original location.
- Metastatic paraganglioma: Cancer has spread to other parts of your body, such as your liver, lungs, bone or distant lymph nodes. Approximately 35% to 50% of malignant paragangliomas may spread to other parts of your body (metastasize).
- Recurrent paraganglioma: Cancer has recurred (come back) after it has been treated. It may come back in the same place or in another part of your body.
How fast does a paraganglioma grow?
Paragangliomas usually grow very slowly. But this could vary from case to case.
Who does paraganglioma affect?
Anyone at any age can get a paraganglioma, but they occur most often in people between 30 and 50 years of age. Approximately 10% of cases occur in children.
How common is paraganglioma?
Paraganglioma is a rare tumor. It’s estimated that only 2 out of every 1 million people have paraganglioma.
Symptoms
When to see a doctor
Complications
- High blood pressure.
- Diabetes.
- Heart failure.
- Some types of heart valve disease.
Prevention
- Control high blood pressure, high cholesterol and diabetes.
- Don't smoke or use tobacco.
- Eat a diet that's low in salt and saturated fat.
- Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
- Get good sleep. Adults should aim for 7 to 9 hours daily.
- Maintain a healthy weight.
- Reduce and manage stress.
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