Paraganglioma: Symptoms,Causes And Treatment

paraganglioma causes


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:

  • 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.


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.


The tests and procedures you might undergo to diagnose a neuroendocrine tumor will depend on where your tumor is located in your body. In general, tests might include:

  • Physical exam. Your doctor may examine your body to better understand your signs and symptoms. He or she may feel for swollen lymph nodes or look for signs that a tumor is producing excess hormones.
  • Tests to look for excess hormones. Your doctor may recommend testing your blood or your urine for signs of excess hormones that are sometimes produced by neuroendocrine tumors.
  • Imaging tests. You might undergo imaging tests, such as ultrasound, CT and MRI, to create pictures of your tumor. For neuroendocrine tumors, pictures are sometimes created using positron emission tomography (PET) with a radioactive tracer that's injected into a vein.
  • Procedures to remove a sample of cells for testing (biopsy). To collect the cells, the doctor might insert a long, thin tube with a light and a camera on the end into your lungs (bronchoscopy), your esophagus (endoscopy) or your rectum (colonoscopy), depending on your situation. Sometimes, collecting a tissue sample requires surgery.

If there's a risk that your neuroendocrine tumor may have spread to other parts of your body, you might have additional tests to determine the extent of the cancer.


The treatment options for your neuroendocrine tumor will depend on the type of tumor, its location, and whether you're experiencing signs and symptoms of excess hormones produced by the tumor.

In general, neuroendocrine tumor treatment options might include:

  • Surgery. Surgery is used to remove the tumor. When possible, surgeons work to remove the entire tumor and some of the healthy tissue that surrounds it. If the tumor can't be removed completely, it might help to remove as much of it as possible.
  • Chemotherapy. Chemotherapy uses strong drugs to kill tumor cells. It can be given through a vein in your arm or taken as a pill. Chemotherapy might be recommended if there's a risk that your neuroendocrine tumor might recur after surgery. It might also be used for advanced tumors that can't be removed with surgery.
  • Targeted drug therapy. Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die. Targeted drug therapy is usually combined with chemotherapy for advanced neuroendocrine tumors.
  • Peptide receptor radionuclide therapy (PRRT). PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance. It allows radiation to be delivered directly to the cancer cells. One PRRT drug, lutetium Lu 177 dotatate (Lutathera), is used to treat advanced neuroendocrine tumors.
  • Medications to control excess hormones. If your neuroendocrine tumor releases excess hormones, your doctor might recommend medications to control your signs and symptoms.
  • Radiation therapy. Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill tumor cells. Some types of neuroendocrine tumors may respond to radiation therapy. It might be recommended if surgery isn't an option.

Other treatments might be available to you depending on your particular situation and your specific type of neuroendocrine tumor.

Coping and support

Being diagnosed with a neuroendocrine tumor can be stressful and overwhelming. With time, you'll find ways to cope with the distress and uncertainty. Until then, you may find that it helps to:

  • Learn enough about your diagnosis to make decisions about your care. Ask your doctor about your neuroendocrine tumor, including your treatment options and, if you like, your prognosis. As you learn more about the type of neuroendocrine tumor you have, you may become more confident in making treatment decisions.
  • Turn to family and friends for support. Stay connected to family and friends for support. It can be tough to talk about your diagnosis, and you'll likely get a range of reactions when you share the news. But talking about your diagnosis and passing along information about your cancer can help you cope — and so can the offers of practical help that often result.
  • Connect with others with neuroendocrine tumors. Consider joining a support group, either in your community or on the internet. A support group of people with the same diagnosis can be a source of useful information, practical tips and encouragement.

Preparing for your appointment

Start by making an appointment with your family doctor if you have any signs or symptoms that worry you. Depending on your particular situation, your doctor may refer you to a specialist.

Because appointments can be brief, and because there's often a lot of information to go over, it's a good idea to be well prepared. Here's some information to help you get ready and know what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, vitamins or supplements that you're taking.
  • Consider taking a family member or friend along. Sometimes it can be difficult to take in all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help make the most of your time together. List questions from most important to least important in case time runs out. For neuroendocrine tumors, some basic questions to ask your doctor include:

  • What type of neuroendocrine tumor do I have?
  • Can you explain what my test results mean?
  • Do you recommend any other tests or procedures?
  • What are my treatment options?
  • What side effects are likely with each treatment?
  • How will treatment affect my daily life?
  • Which treatment options do you think are best for me?
  • How quickly must I make a decision on my treatment?
  • Should I get a second opinion? What will that cost, and will my insurance cover it?
  • Are there brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions that occur to you.

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may allow time later to cover other points you want to address. Your doctor may ask:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?