A paraganglioma is an abnormal growth of cells that forms from a specific type of nerve cell that's found throughout the body. These specific nerve cells (chromaffin cells) perform important functions in the body, including regulating blood pressure.
When chromaffin cells become abnormal they can form growths (tumors). When the tumors happen in the adrenal glands they're called pheochromocytomas. When the tumors happen elsewhere in the body they're called paragangliomas.
Paragangliomas are usually noncancerous (benign). But some paragangliomas can become cancerous (malignant) and spread (metastasize) to other parts of the body.
Paragangliomas are rare tumors. They can occur at any age, but they're most often diagnosed in adults between 30 and 50. Most paragangliomas have no known cause, but some are caused by gene mutations that are passed from parents to children.
Paraganglioma cells commonly secrete hormones known as catecholamines or adrenaline, which is the fight-or-flight hormone. This can cause episodes of high blood pressure, a rapid heartbeat, sweating, headache and tremors.
Paraganglioma treatment most often involves surgery to remove the tumor. If the paraganglioma is cancerous or spreads to other areas of the body, additional treatments may be considered.
Tests used to diagnosed paraganglioma include:
Paragangliomas are rare, and many doctors have only rarely, if ever, cared for a person with this diagnosis. Ask your doctor about his or her experiencing treating paraganglioma. If you feel uncomfortable about your doctor's experience with this rare condition, consider seeking a second opinion from a doctor who specializes in caring for people with paragangliomas and other neuroendocrine tumors.
Your treatment options will depend on where your paraganglioma is located, whether it has spread to other areas of the body, and whether it's producing excess hormones that cause signs and symptoms.
Paraganglioma treatment usually involves surgery. If the paraganglioma cells produce hormones, it's necessary to block the hormones before proceeding with treatment. Usually this is done with medications. If the paraganglioma can't be removed completely during an operation or if it spreads to other parts of the body, additional treatments may be considered.
Treatment options include:
Treatments to control hormones produced by the tumor. If your paraganglioma produces excess hormones, you may need treatments to reduce hormone levels and control signs and symptoms. It's necessary to lower and control the hormone levels before you begin paraganglioma treatment.
Medications used to control hormone levels include alpha blockers, beta blockers and calcium channel blockers. Other measures might include eating a diet high in sodium and drinking plenty of fluids.
Surgery. Surgery is used to remove the paraganglioma. Even if the paraganglioma can't be removed completely, your doctor may recommend an operation to remove as much as possible.
If your paraganglioma produces excess hormones, you may need to take medications before surgery to control the hormones. When paragangliomas that produce hormones are disturbed, such as during surgery, excess hormones can be released and cause serious problems.
Radiation therapy. Radiation therapy uses high-energy beams, such as X-rays and protons, to control tumor growth. Radiation therapy might be recommended if the paraganglioma can't be removed completely with surgery. It can also be used to relieve pain caused by a paraganglioma that spreads to other parts of the body.
One specialized type of radiation therapy called stereotactic body radiotherapy involves aiming many beams of radiation at the tumor. The beams are precisely aimed on the paraganglioma cells so that there's minimal risk of damage to nearby healthy tissue.
Thermal ablation therapy. Thermal ablation treatment uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This might be an option in certain situations, such as when a paraganglioma spreads to other areas of the body, such as the bones or the liver.
One type of thermal ablation treatment called radiofrequency ablation uses electrical energy to heat the tumor cells. Another type of treatment called cryoablation uses cold gas to freeze the tumors cells.
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