Paraganglioma is a type of neuroendocrine tumor that forms near certain blood vessels and nerves outside of the adrenal glands. The adrenal glands are important for making hormones that control many functions in the body and are located on top of the kidneys. The nerve cells involved in paraganglioma are part of the peripheral nervous system, meaning the part of the nervous system outside of the brain and spinal cord. These tumors can also be called extra-adrenal pheochromocytomas.

Approximately 35-50% of paragangliomas may spread to other parts of the body.

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).

Paraganglioma is rare and it is estimated that only 2 people out of every 1 million people have paraganglioma. It is most often found in people aged 30 to 50 years old.

Scientists have found mutations in approximately 20 different genes that they think may lead to pheochromocytoma and paraganglioma. Mutations in the genes RET, VHL, NF1, SDHA, SDHB, SDHC, SDHD, SDHAF2, MDH2, IDH1, PHD1/PHD2, HIF2A/EPAS1/2, TMEM127, MAX, HRAS, MAML3 and CSDE1 may play a role in forming pheochromocytoma and paraganglioma.

Scientists have found that some genetic conditions may be associated with having paraganglioma. These genetic conditions include:
• Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B)
• von Hippel-Lindau (VHL) syndrome
• Neurofibromatosis type 1 (NF1)
• Hereditary paraganglioma syndrome
• Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST])
• Carney triad (paraganglioma, GIST, and pulmonary chondroma)

Yes, paraganglioma can run in families, but not always. Some of these inherited cases may be associated with a genetic condition, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis type 1.

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. 

The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:

• Where the tumor is in your body
• If the cancer has spread to other parts of your body
• How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis.

Patients with a small paraganglioma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with paraganglioma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.