Paraganglioma

Overview

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, including 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.

Diagnosis

Tests used to diagnosed paraganglioma include:

• Blood and urine tests. Your doctor may recommend blood tests and urine tests to measure the levels of hormones in your body. These tests may detect extra hormones produced by paraganglioma cells or a tumor marker called chromogranin A.
• Imaging tests. Imaging tests create pictures of the paraganglioma to help your doctor better understand the extent of the tumor and help determine the best treatment options. Tests may include MRI, CT, and specialized nuclear medicine imaging such as a metaiodobenzylguanidine (MIBG) scan and a positron emission tomography (PET) scan.
• Genetic testing. Paragangliomas are sometimes caused by gene mutations that can be passed from parents to children. Genetic testing may be recommended as part of your care.

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.

Treatment

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.

• Chemotherapy. Chemotherapy uses drugs to destroy fast-growing cells, such as cancer cells. If your paraganglioma has spread, your doctor may recommend chemotherapy to help shrink the tumors. If your paraganglioma produces excess hormones, you'll receive medications to control the hormone levels before undergoing chemotherapy.
• Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can slow the growth of cancer cells or cause them to die.
• Drugs that deliver radiation more directly to tumor cells. Drugs that combine a chemical that seeks out paraganglioma cells with a radioactive substance can deliver radiation directly to the tumor cells. These treatments might be an option for a paraganglioma that spreads to other parts of the body.
• Clinical trials. Clinical trials are studies of new treatments or new ways of using existing treatments. If you're interested in clinical trials, talk with your doctor about your options. Together you can consider the benefits and risks of experimental treatments.
• Watchful waiting. In certain situations, such as if the paraganglioma is slow growing and isn't causing signs and symptoms, your doctor may recommend against immediate treatment. Instead, your doctor may monitor your condition during regular follow-up appointments.