Paragangliomas are tumors originating from neuronal tissue; they were formerly called extra-adrenal pheochromocytomas. There are developmentally two subgroups of these tumors: parasympathetic paragangliomas and sympathetic paragangliomas. These subgroups differ in the type of tissue from which they form (parasympathetic versus sympathetic) and also in their location and hormonal production. Parasympathetic tissue is important for certain body processes, including salivation, urination, and digestion. Sympathetic tissue forms the tissue important for "fight-or-flight" responses.

The group of paragangliomas that develop from parasympathetic-associated tissue in the head and neck are usually referred to as "head and neck paragangliomas." These tumors can be locally invasive but usually do not metastasize or produce catecholamines (stress hormones). Signs and symptoms of head and neck paragangliomas are usually due to the tumor mass itself instead of the secreted catecholamines.

Paragangliomas that develop from sympathetic neuronal tissue are usually localized in the chest, abdomen, or pelvis. These tumors often have excessive hormone secretion, which makes them very similar to pheochromocytomas. As a result of excessive hormone secretion, paragangliomas often cause the signs and symptoms described below (e.g., heart palpitations, irregular heartbeat, hypertension, and sweating). Sympathetic (or extra-adrenal) paragangliomas generally tend to be more malignant than pheochromocytomas (localized to the adrenal gland).

The exact cause of glomus jugulare tumors is unknown. Most glomus jugulare tumors develop by chance in individuals who have no family history of this condition. However, in some families, multiple relatives are affected by glomus jugulare tumors, which indicates there may be an inherited risk factor (genetic predisposition) that increases the chance of developing this disease. Changes (mutations) in the SDHD gene have been identified as an inherited risk factor in some glomus jugulare tumors.

Some people with paraganglioma have symptoms, but others don’t. Symptoms can include:

• High blood pressure
• Fast heartbeat
• Sweating
• Headache
• Shaking or tremors

Lab Tests: If you have symptoms of paraganglioma, your doctor will order lab tests of your urine and blood to check your hormone levels.

Imaging: Your doctor will use imaging scans such as MRI, CT, and PET to look at where the tumor is and how big it is. They will also check for signs that the tumor has spread to other parts of the body.

Treatment of paraganglioma may involve many different doctors, including doctors who specialize in hormone disorders and doctors who diagnose and treat neuroendocrine tumors. Treatment options to discuss with your doctor include:

Medications: Your doctor may give you medications to control your symptoms, such as alpha blockers and may be followed by beta blockers, which are drugs to control high blood pressure.

Watch and wait: In some cases, the tumor grows very slowly. In this case it may be safest for your doctor to check your tumor regularly without treating it.

Surgery: Once paraganglioma is diagnosed, you may have surgery to remove the tumor. Sometimes surgery is not an option, in which case, your doctor will discuss other options with you.

Radiation therapy: Radiation therapy can be used to slow the tumors from growing and to help relieve symptoms.

It is important to talk with a team of specialists to decide what the right treatment is for you.