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MyPART - My Pediatric and Adult Rare Tumor Network
 

Paraganglioma

What is paraganglioma? 

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. 

How common is paraganglioma?

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. 

How is paraganglioma diagnosed?

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.

How is paraganglioma treated?

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. You can contact MyPART to get help finding specialists.

Does paraganglioma run in families?

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. 

How does paraganglioma form?

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)

What is the prognosis for someone with paraganglioma?

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%.