Definition

What is Paraganglioma?

Paraganglioma is a pathological condition in which there is development of a type of tumor in the peripheral nervous system of the body. The peripheral nervous system is further subdivided into sympathetic and parasympathetic nervous systems. The function of the peripheral nervous system is controlling various bodily functions to include the heart rate, blood pressure, urination, and intestinal motions.

Paraganglioma developing in peripheral nervous system may cause alterations in many bodily functions like the blood pressure, urination, heart rate, etc. Paraganglioma can develop anywhere along the peripheral nervous system but they are mainly found in the abdomen and are related to some form of tumors of the adrenal glands called phaeochromocytoma.

How common is Paraganglioma?

Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Paraganglioma?

The common symptoms of Paraganglioma are:

  • Persistent elevated high blood pressure
  • Increased heart rate
  • Persistent headaches
  • Facial flushing and sweating
  • Severe anxiety and panic attacks

Some of the less common symptoms of Paraganglioma are:

  • Pale skin
  • Blurred vision
  • Unintentional weight loss
  • Constipation
  • Abdominal pain
  • Hyperglycemia
  • Other psychiatric abnormalities

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you or your loved one has any signs or symptoms listed above or you have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Paraganglioma?

In some cases, the primary causes of paragangliomas are attributed to hereditary syndromes that may affect other organs. If the tumors have no association with any syndrome, they are referred to as nonsyndromic paragangliomas. Genetic mutations occur in both syndromic and nonsyndromic paragangliomas.

Both syndromic and nonsyndromic paraganglia are associated with mutations in the genes VHL, RET, SDHA, SDHB and SDHD. The genes involved with nonsyndromic paragangliomas perform important functions in managing proteins that regulate cell division and blood vessel formation. Mutations can be passed from just one parent with the altered gene and allow uncontrolled cell development which can result in tumors.

Some people develop nonsyndromic paragangliomas without having a mutation in the genes identified with the condition. However, doctors believe this may be due the existence of other gene mutations that have yet to be identified.

A number of inherited syndromes also are related to the development of paragangliomas. Some of these gene-altering conditions include:

  • Hereditary paraganglioma syndrome
  • Multiple endocrine neoplasia 2, types a and b (men2a and men2b)
  • Neurofibromatosis type 1 (nf1)
  • Von hippel-lindau syndrome (vhl)
  • Carney-stratakis dyad (paraganglioma with gastrointestinal stomal tumor, or gist)
  • Carney triad (pulmonary chondroma, GIST and paraganglioma)

Risk factors

What increases my risk for Paraganglioma?

Please consult with your doctor for further information.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Paraganglioma diagnosed?

If Paraganglioma is suspected, you need to go through a battery of tests in order to confirm the disease condition.

The doctor may begin with collecting urine over a period of 24 hours and sending it for analysis to look for presence of any excessive adrenaline or noradrenaline secretions.

Simultaneously blood tests may also be done for the same reason.

Once this study is done a CT and MRI scan will be done, especially of the abdominal area to identify presence of any tumors.

In some cases a PET scan may also be done to look for presence of Paraganglioma and whether the tumor has spread to any degree.

Genetic testing may also be done to check for any gene defects in case if there is a family history of Paraganglioma.

How is Paraganglioma treated?

Treatment of a paraganglioma depends on where the abnormal growth is located and whether it is cancerous. A paraganglioma is considered cancerous (malignant) if it spreads to other parts of the body (metastasizes). This is a rare occurrence in paragangliomas.

Cells from a cancerous (malignant) paraganglioma can migrate to lymph nodes, bone, liver, lungs or elsewhere. In people with this type of paraganglioma, it’s critical to treat any elevated hormone levels and other signs and symptoms.

Treatment options include:

Medications. Your doctor will first attempt to control symptoms caused by your paraganglioma, such as high blood pressure, if you have the type of paraganglioma that produces hormones. Drugs that usually help people with this condition include alpha blockers and beta blockers. In people with hormone-producing paragangliomas, it’s critical to control blood pressure before surgery or other therapy.

Surgery. Surgery to remove the tumor is generally the first choice for treatment, if feasible. Even if the tumor has spread, surgery is often used to reduce its size.

If paraganglioma tissue that produces hormones is disturbed, as happens during surgery, hormones in the tumor can be released and cause serious problems, such as elevated blood pressure and pulse. To prevent that, your doctor will have you prepare for surgery by taking medication to block the hormone’s effects for 7 to 14 days before surgery. You may also be asked to record your blood pressure multiple times a day, to follow a high-sodium diet and to report your blood pressure results to your medical team.

Paraganglioma surgery is done under general anesthesia. It’s important that specialized surgeons and anesthesiologists be involved. You’ll likely need to stay in the hospital for several days after surgery. Even after successful removal of the tumor during surgery, tumors may recur. So you’ll need to visit your doctor regularly for follow-up appointments.

Radiation therapy. Radiation therapy is sometimes used to help control tumor growth and improve your symptoms if other options, such as surgery, aren’t advisable. If the tumor is in the head or near vital nerves or blood vessels, a technique called stereotactic body radiation therapy may be used to limit damage to nearby healthy tissue.

Thermal ablation therapy. Under some circumstances, specialized interventional radiologists may destroy tumor deposits with an approach called thermal ablation therapy.

Clinical trials. You may be eligible for a clinical trial testing an experimental treatment.

Watchful waiting. If the tumor is slow growing, your doctor may monitor your condition during regular follow-up appointments even if you aren’t undergoing therapy.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Paraganglioma?

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: February 5, 2018 | Last Modified: February 5, 2018

Want to live your best life?
Get the Hello Doktor Daily newsletter for health tips, wellness updates and more.