Description

A paraganglioma is a growth of cells that can occur in different places in the body. The growth, called a tumor, forms an important type of nerve cells that are found throughout the body. Paragangliomas most often begin in the head, the neck, the area of the stomach or pelvis.

A paraganglioma is rare. And most of the times, is that it is not cancer. When the tumor is not cancer is called benign. Sometimes a paraganglioma is cancerous. A cancerous tumor can spread to other parts of the body.

Paragangliomas often do not have a clear cause. Some of the paragangliomas are caused by changes in the DNA that are passed from parents to children.

Paragangliomas can form at any age. The health care professionals most often found in adults between the ages of 20 and 50.

Paraganglioma treatment usually involves surgery to remove the tumor. If the paraganglioma is cancerous and spread to other areas of the body, more treatments may be necessary.

Symptoms

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

The symptoms of a paraganglioma in the head or neck may include:

  • A rhythmic pulsation or whooshing sound in the ears, called pulsatile tinnitus.
  • Difficulty swallowing.
  • The hoarse voice.
  • The hearing loss.
  • Blurred vision.
  • The dizziness.

The symptoms of a paraganglioma in the head and neck could happen as the tumor grows. The tumor can exert pressure on nearby structures.

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

The symptoms of paragangliomas that make the hormones include:

  • The high blood pressure.
  • Feelings of having a fast heartbeat, palpitations or pounding heart.
  • The sudden loss of color in the face.
  • Sweating.
  • The pain of a headache.
  • Uncontrollable shaking of the hands or arms.
  • The General weakness.

These symptoms can appear and disappear.

Some people with paragangliomas do not have any symptoms. You can learn these tumors when the imaging tests performed for other reasons happen to spot tumors.

When to see a doctor

Make an appointment with a doctor or other health care professional if you are concerned that you may have paraganglioma symptoms. This is important if you have multiple paraganglioma symptoms at the same time.

Talk with your healthcare provider about your risk of paraganglioma if you have high blood pressure that is difficult to control. This includes high blood pressure that needs treatment with more than one drug. Also talk with your health care professional if your blood pressure spikes once in a while or a professional of the health measures.

Causes

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

A paraganglioma is a growth of cells. It is formed from a type of nerve cell is called one of the chromaffin cells. Chromaffin cells perform a key role in the body, including the control of blood pressure.

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

The majority of paragangliomas stay where they started. They do not spread to other parts of the body. But sometimes, the cells can get out of a paraganglioma and propagation. When this happens, it is called metastatic paraganglioma. When a paraganglioma spreads, it most often spreads to the lymph nodes nearby. It can also spread to the lungs, liver and bones.

A paraganglioma is closely associated with another rare tumor called a pheochromocytoma. A pheochromocytoma is a tumor that begins in the 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 family history of this type of tumor. Some of the paragangliomas are caused by changes in the DNA that are passed from parents to children. Having a family history of paraganglioma could be a sign that certain changes in the DNA in your family.

Some other health conditions caused by changes in the DNA that is passed from parents to children increase the risk of paragangliomas. These conditions include:

  • Multiple endocrine Neoplasia type 2. Multiple endocrine Neoplasia type 2, also called of MEN 2, can cause tumors in one or more of the glands that produce hormones, including the thyroid and parathyroid glands. There are two types of MEN 2 — type 2A and type 2B. Both increase the risk of paragangliomas.
  • The Von Hippel-Lindau disease. The Von Hippel-Lindau disease can cause tumors and cysts are formed in many parts of the body. Possible sites include the brain, spinal cord, and kidneys.
  • The Neurofibromatosis 1. Neurofibromatosis 1 causes tumors called neurofibromas that form in the skin. The condition can also cause tumors in the optic nerve. The optic nerve is the nerve in 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 of a paraganglioma.
  • Carney-Stratakis dyad. Carney-Stratakis dyad causes of digestive tract tumors and paragangliomas.

Diagnosis

Paraganglioma diagnosis often begins with blood and urine tests. These tests can look for signs that a tumor is doing extra hormones. Other tests may include imaging tests and genetic testing.

Blood and urine tests

Blood and urine tests can measure the levels of hormones in the body. It can detect extra catecholamines, hormones produced by a paraganglioma. Or you can find other clues of a paraganglioma as a protein called chromogranin A.

Imaging tests

Your health care professional may recommend imaging tests if your symptoms, a family history, or blood and urine tests suggest that there may be a paraganglioma. These pictures can show the location and size of the tumor. You can also help guide your treatment options.

The following imaging tests may be used to paraganglioma:

  • Magnetic resonance imaging, also called magnetic resonance imaging (mri uses radio waves and a magnetic field to produce detailed images.
  • Computerized axial tomography scan, also called a ct scan combines a series of X-ray images taken from different angles around the body.
  • Metaiodobenzylguanidine scan, also called MIBG scan, which detects the injection of a radioactive tracer that is absorbed by the paragangliomas.
  • The positron emission tomography scan, also called a PET, we also detect a radioactive tracer absorbed by a tumor.

Genetic testing

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

Their parents, children or siblings can also check the changes in the DNA that increase the risk of paragangliomas. Your health care team can refer you to a genetic counselor or other health care professional trained in genetics. This person can help you and your loved ones to decide whether to get genetic testing.

Treatment

More often, paraganglioma treatment consists of surgery to remove the tumor. If the tumor produces hormones, health professionals often use drugs to block the hormones in the first place. 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 several factors. These include:

  • Where the tumor is located.
  • If it is cancerous and has spread to other parts of the body.
  • If we produce more hormones that cause symptoms.

The treatment options include:

Treatments for the control of the hormones produced by the tumor

If your paraganglioma makes extra catecholamines, it is likely that you will need treatment to block the effects or decrease the levels of these hormones. These treatments for lowering high blood pressure and the control of other symptoms. It is important that the blood pressure and symptoms of being under control before other paraganglioma treatment is initiated. That is because the treatment can cause the tumor to release of large amounts of catecholamines and that can lead to serious problems.

Medications used to control the effects of catecholamines, which include some blood pressure medications. These drugs include alpha-blockers, beta-blockers and calcium channel blockers. Other measures may include the consumption of a diet high in sodium, and drinking plenty of fluids.

Surgery

The surgery may be done to remove a paraganglioma. Even if a paraganglioma can't be removed completely, your health care professional may recommend surgery to remove as much of the tumor as possible.

The type of surgery that is used to remove a paraganglioma depends on where you are located. The tumor location also determines the type of surgeon that performed the procedure. For example:

  • Tumors in the head and neck can be treated by surgeons of the head and neck.
  • Tumors that affect the brain, spine, and nerves can be treated by neurosurgeons.
  • Tumors that affect the hormones by the glands can be treated by endocrine surgeons.
  • Tumors that affect the blood vessels can be treated by vascular surgeons.

Sometimes surgeons of different specialties to work together for paraganglioma of the surgery.

Radiation therapy

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

Sometimes a special type of radiation called stereotactic radiosurgery is used to treat the paragangliomas of the head and neck area. This type of radiation is the goal of many rays of energy in the tumor. Each beam of light is not very powerful. But the point where the beams meet receives 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 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 drugs. If your paraganglioma has spread, your health care professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma produces more hormones, you will receive medications for the control of hormone levels before starting chemotherapy.

Targeted therapy

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

Peptide receptor radionuclide therapy

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

A medication that works this way is the lutetium-177 Lu-dotatate (Lutathera). Can be used when surgery is not 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 old treatments. If you are interested in clinical trials for the paraganglioma, talk with your health care professional about your options. Together you can weigh the benefits and risks of the treatments that researchers are studying.

Watchful waiting

Sometimes, health professionals recommend against starting paraganglioma treatment immediately. Instead, they prefer see closely her condition with regular health care routine. This is known as watchful waiting. For example, expectant management may be an option if a paraganglioma grows slowly and does not cause symptoms.

Preparing for your appointment

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

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

What you can do

When you make the appointment, ask if there is something that you need to do beforehand. For example, you may need to stop eating a certain number of hours before a test. Also make a list of:

  • Its symptoms, including those that do not seem to be related to the reason for your appointment.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All medications, vitamins or supplements that you are taking, including the dosage.
  • Questions to ask your health care professional.

Have a family member or friend if you can. This person can help you remember the information they give you.

Some basic questions to ask your health care professional include:

  • What is likely causing my symptoms? There are other possible causes of the symptoms?
  • What tests do I need?
  • Is my condition likely to be short-term or ongoing?
  • What treatment do you recommend it? There are other treatment options apart from the main that I have suggested?
  • I have other health conditions. How can I best manage them together?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Feel free to ask questions.

What to expect from your doctor

Your healthcare provider will likely ask questions such as:

  • When did the symptoms begin?
  • The symptoms been constant or come and go?
  • How bad are the symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Do you have a family history of paragangliomas or any genetic conditions?
Symptoms and treatment of Paraganglioma