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Overview

A paraganglioma is a growth of cells that can happen in different places in the body. The growth, called a tumor, forms from an important type of nerve cell that's found throughout the body. Paragangliomas most often start in the head, neck, stomach area or pelvis.

A paraganglioma is rare. And most often, it's not cancer. When a tumor isn't cancer, it's called benign. Sometimes a paraganglioma is cancerous. A cancerous tumor can spread to other parts of the body.

Paragangliomas often don't have a clear cause. Some paragangliomas are caused by DNA changes that pass from parents to children.

Paragangliomas can form at any age. Healthcare professionals most often find them in adults between the ages of 20 and 50.

Paraganglioma treatment typically involves surgery to remove the tumor. If the paraganglioma is cancerous and spreads to other areas of the body, more treatments may be needed.

Symptoms

Paraganglioma symptoms may depend on where the tumor starts. Paragangliomas most often start in the head, neck, stomach area or pelvis.

Symptoms of a paraganglioma in the head or neck can include:

• A rhythmic pulsing or whooshing sound in the ears, called pulsatile tinnitus.
• Trouble swallowing.
• Hoarse voice.
• Hearing loss.
• Blurred vision.
• Dizziness.

Symptoms of a paraganglioma in the head and neck area might happen as the tumor grows larger. The tumor can press on nearby structures.

When paragangliomas form in other places in the body, the symptoms are more likely to be caused by hormones that the paraganglioma makes. The hormones, called catecholamines, play a role in how the body response to stress. They include adrenaline, also known as the fight-or-flight hormone.

Symptoms of paragangliomas that make hormones include:

• High blood pressure.
• Feelings of having a fast-beating, fluttering or pounding heart.
• The sudden loss of color in the face.
• Sweating.
• Headache.
• Uncontrolled shaking in the hands or arms.
• General weakness.

These symptoms may come and go.

Some people with paragangliomas don't have any symptoms. They may learn they have these tumors when imaging tests done for other reasons happen to spot the tumors.

When to see a doctor

Make an appointment with a doctor or other healthcare professional if you're concerned you may have paraganglioma symptoms. This is important if you have multiple paraganglioma symptoms at the same time.

Talk with your healthcare professional about your risk of paraganglioma if you have high blood pressure that's hard to control. This includes high blood pressure that needs treatment with more than one medicine. Also talk with your healthcare professional if your blood pressure spikes from time to time when you or a healthcare professional measures it.

Causes

Paragangliomas often don't have a clear cause. Sometimes these tumors run in families. They can be caused by DNA changes that are passed from parents to children. But many people with paragangliomas don't have a family history of these tumors and the cause is not known.

A paraganglioma is a growth of cells. It forms from a type of nerve cell called a chromaffin cell. Chromaffin cells perform key roles in the body, including controlling blood pressure.

A paraganglioma starts when chromaffin cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions also tell the cells to die at a set time. In paraganglioma cells, the DNA changes give different instructions. The changes tell the paraganglioma cells to make many more cells quickly. The cells keep living when healthy cells would die. This causes a growth of cells called a tumor.

Most paragangliomas stay where they started. They don't spread to other parts of the body. But sometimes cells can break away from a paraganglioma and spread. When this happens, it's called metastatic paraganglioma. When a paraganglioma spreads, it most often spreads to nearby lymph nodes. It also can spread to the lungs, liver and bones.

A paraganglioma is closely related to another rare tumor called a pheochromocytoma. A pheochromocytoma is a tumor that starts in chromaffin cells in the adrenal glands. The adrenal glands are two glands that sit on top of the kidneys.

Risk factors

The risk of paraganglioma is higher in people who have a family history of this tumor. Some paragangliomas are caused by DNA changes that pass from parents to children. Having a family history of paraganglioma might be a sign that certain DNA changes run in your family.

Some other health conditions caused by DNA changes that pass from parents to children raise the risk of paragangliomas. These conditions include:

• Multiple endocrine neoplasia, type 2. Multiple endocrine neoplasia, type 2, also called MEN 2, can cause tumors in one or more of the glands that make hormones, including the thyroid and the parathyroid glands. There are two types of MEN 2 — type 2A and type 2B. Both raise the risk of paragangliomas.
• Von Hippel-Lindau disease. Von Hippel-Lindau disease can cause tumors and cysts to form in many parts of the body. Possible sites include the brain, spinal cord and kidneys.
• Neurofibromatosis 1. Neurofibromatosis 1 causes tumors called neurofibromas to form in the skin. The condition also can cause tumors of the optic nerve. The optic nerve is the nerve at the back of the eye that connects to the brain.
• Hereditary paraganglioma syndromes. Hereditary paraganglioma syndromes can cause pheochromocytomas or paragangliomas. People with these syndromes often have more than one paraganglioma.
• Carney-Stratakis dyad. Carney-Stratakis dyad causes tumors of the digestive tract and paragangliomas.

Diagnosis

Paraganglioma diagnosis often starts with blood and urine tests. These tests can look for signs that a tumor is making extra hormones. Other tests might include imaging tests and genetic tests.

Blood and urine tests

Blood and urine tests can measure the levels of hormones in the body. They may detect extra catecholamine hormones made by a paraganglioma. Or they may find other clues of a paraganglioma such as a protein called chromogranin A.

Imaging tests

Your healthcare professional may recommend imaging tests if your symptoms, family history, or blood and urine tests suggest you may have a paraganglioma. These images can show the location and size of the tumor. They also may help guide your treatment options.

The following imaging tests may be used for paraganglioma:

• Magnetic resonance imaging, also called MRI, uses radio waves and a magnetic field to make detailed images.
• Computerized tomography scan, also called CT scan, combines a series of X-ray images taken from various angles around the body.
• Metaiodobenzylguanidine scan, also called MIBG scan, detects an injected radioactive tracer that's absorbed by paragangliomas.
• Positron emission tomography scan, also called PET scan, also detects a radioactive tracer absorbed by a tumor.

Genetic testing

Some DNA changes passed from parents to children make paragangliomas more likely to form. If you have a paraganglioma, your healthcare professional may recommend genetic testing to look for these DNA changes in your body. Results of genetic testing can help predict the chances of your tumor coming back after treatment.

Your parents, children or siblings also can be checked for DNA changes that raise the risk of paragangliomas. Your healthcare team may refer you to a genetic counselor or another healthcare professional trained in genetics. This person can help you and your loved ones decide whether to get genetic testing.

Treatment

Most often, paraganglioma treatment involves surgery to remove the tumor. If the tumor makes hormones, healthcare professionals often use medicines to block the hormones first. If the paraganglioma can't be removed with surgery or if it spreads, you may need other treatments.

Your treatment options for paraganglioma depend on various factors. These include:

• Where the tumor is located.
• Whether it is cancerous and has spread to other parts of the body.
• Whether it makes extra hormones that cause symptoms.

Treatment options include:

Treatments to control hormones made by the tumor

If your paraganglioma makes extra catecholamines, you'll likely need treatments to block the effects or lower the levels of these hormones. These treatments lower high blood pressure and control other symptoms. It's important for blood pressure and symptoms to be under control before other paraganglioma treatment starts. That's because treatment can cause the tumor to release very large amounts of catecholamines and that can lead to serious problems.

Medicines used to control catecholamine effects include some blood pressure medicines. These medicines include alpha blockers, beta blockers and calcium channel blockers. Other measures may include eating a diet high in sodium and drinking plenty of fluids.

Surgery

Surgery can be done to remove a paraganglioma. Even if a paraganglioma can't be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible.

The kind of surgery that's used to remove a paraganglioma depends on where it's located. The tumor's location also determines the kind of surgeon who does the procedure. For example:

• Tumors in the head and neck area might be treated by head and neck surgeons.
• Tumors that affect the brain, spine and nerves might be treated by neurosurgeons.
• Tumors that affect hormone-making glands might be treated by endocrine surgeons.
• Tumors that affect blood vessels might be treated by vascular surgeons.

Sometimes surgeons from different specialties work together during paraganglioma surgery.

Radiation therapy

Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can't be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body.

Sometimes a special kind of radiation called stereotactic radiosurgery is used to treat paragangliomas in the head and neck area. This kind of radiation aims many beams of energy at the tumor. Each beam isn't very powerful. But the point where the beams meet gets a large dose of radiation to kill the tumor cells.

Ablation therapy

Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body.

Radiofrequency ablation uses electrical energy to heat the tumor cells. Another type of ablation called cryoablation uses cold gas to freeze the tumor cells.

Chemotherapy

Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

Targeted therapy

Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn't an option. Targeted therapy also may be used if the tumor spreads to other parts of the body.

Peptide receptor radionuclide therapy

Peptide receptor radionuclide therapy, also called PRRT, uses medicine to give radiation directly to tumor cells. The medicine combines a substance that finds tumor cells with a substance that contains radiation. The medicine is given through a vein. The medicine goes through the body and sticks to paraganglioma cells. Over days to weeks, the medicine delivers radiation directly to the tumor cells.

One medicine that works in this way is lutetium Lu 177 dotatate (Lutathera). It might be used when surgery isn't an option or when paraganglioma spreads to other parts of the body.

Clinical trials

Clinical trials are studies of new treatments or new ways of using older treatments. If you're interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying.

Watchful waiting

Sometimes, healthcare professionals recommend against starting paraganglioma treatment right away. Instead, they may prefer to closely watch your condition with regular healthcare checkups. This is known as watchful waiting. For example, watchful waiting may be an option if a paraganglioma grows slowly and doesn't cause symptoms.

Preparing for an appointment

Start by making an appointment with a doctor or other healthcare professional if you have any symptoms that worry you. If your health professional suspects you might have paraganglioma, that person may refer you to a specialist. This specialist may be a doctor who treats conditions that affect the body's hormones, called an endocrinologist.

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance. For instance, you may need to stop eating a certain number of hours before a test. Also make a list of:

• Your symptoms, including any that don't seem related to the reason for your appointment.
• Key personal information, including major stresses, recent life changes and family medical history.
• All medicines, vitamins or other supplements you take, including the doses.
• Questions to ask your healthcare professional.

Take a family member or friend along if you can. This person can help you remember the information you're given.

Some basic questions to ask your healthcare professional include:

• What's likely causing my symptoms? Are there other possible causes for my symptoms?
• What tests do I need?
• Is my condition likely short term or ongoing?
• What treatment do you recommend? Are there other treatment options aside from the main one you've suggested?
• I have other health conditions. How can I best manage them together?
• Are there restrictions I need to follow?
• Should I see a specialist?
• Are there brochures or other printed material I can have? What websites do you recommend?

Feel free to ask other questions.

Treatment

What to expect from your doctor

Your healthcare professional is likely to ask you questions such as:

• When did your symptoms start?
• Have your symptoms been constant, or do they come and go?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?
• Do you have a family history of paragangliomas or any genetic conditions?