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Paragangliomas form in nerve tissue in the adrenal glands and near certain blood vesselsand nerves. Paragangliomas that form in the adrenal glands are called pheochromocytomas. Paragangliomas that form outside the adrenal glands are called extra-adrenal paragangliomas. In this summary, extra-adrenal paragangliomas are called paragangliomas.Pheochromocytomas and paragangliomas may be benign (not cancer) or malignant(cancer).
Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.Pheochromocytoma is a rare tumour of the adrenal medulla. Usually, pheochromocytoma affects one adrenal gland, but it may affect both adrenal glands. Sometimes there is more than one tumour in one adrenal gland.The adrenal glands make important hormones called catecholamines. Adrenaline(epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Sometimes a pheochromocytoma will release extra adrenaline and noradrenaline into the blood and cause signs or symptoms of disease.
Paraganglioma of the head and neck. A rare tumour that often forms near the carotid artery. It may also form along nerve pathways in the head and neck and in other parts of the body.
Paragangliomas are rare tumours that form near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of these extra catecholamines into the blood may cause signs or symptoms of disease.
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor doesn't mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk to your doctor if you think you may be at risk.
The following inherited syndromes or gene changes increase the risk of pheochromocytoma or paraganglioma:
Some tumours do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumours are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have any of the following:
The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death.
Signs and symptoms of pheochromocytoma and paraganglioma may occur when one of the following events happens:
The following tests and procedures may be used:
All patients who are diagnosed with pheochromocytoma or paraganglioma should have genetic counseling to find out their risk for having an inherited syndrome and other related cancers.
Genetic testing may be recommended by a genetic counselor for patients who:
Genetic testing is sometimes recommended for patients with pheochromocytoma who:
When certain gene changes are found during genetic testing, the testing is usually offered to family members who are at risk but do not have signs or symptoms.
Genetic testing is not recommended for patients older than 50 years.
The prognosis (chance of recovery) and treatment options depend on the following:
For more information on Paraganglioma click here
This link is to the National Cancer Institute (NCI) cancer website in the United States. There may be references to drugs and clinical trials that are not available here in Australia.
Page last updated: 06/05/2020