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How Common Is Paraganglioma Or Is It A Rare Disease?

Paraganglioma is a rare slow-growing tumor found in 2 out of one million people every year and the prevalence of pheochromocytoma is 1 in 500,000. 1.

Paraganglioma is most often found in people aged 30 to 50 years old and affects less than 200,000 people in the US population. 2.

Paraganglioma is recorded as a rare disease by the Office of Rare Diseases (ORD) of the National Institutes of Health. 3. 4.

How Common Is Paraganglioma?

Paraganglioma is an inherited condition characterized by a group of cells found in the nerve cells called ganglia in the autonomic nervous system. The condition mostly occurs in the head and the neck that begins in the chromaffin cells of the adrenal medulla. Most people develop a pheochromocytoma in one of the adrenal glands however in cases it can occur in both the glands.

Paraganglioma is a rare slow-growing tumor found in 2 out of one million people every year and the prevalence of pheochromocytoma is 1 in 500,000. High blood pressure is the typical symptom of paraganglioma and occurs in less than 0.2% of people with high blood pressure. 1.

Paraganglioma is most often found in middle-aged people and affects less than 200,000 people in the US population. Nearly 10% of the cancer is noticed in young adults and children and the survival rate is approximately 5 years from the time of diagnosis.

However, survival often depends on several factors that include the location and the stage of the tumor. The prevalence of the condition includes syndromic and non-syndromic paraganglioma and pheochromocytoma however paraganglioma is a rare condition when compared to pheochromocytoma. 2.

Is Paraganglioma A Rare Disease?

Paraganglioma is recorded as a rare type of tumor by the Office of Rare Diseases (ORD) of the National Institutes of Health. It arises from chromaffin cells that produce hormones for the proper functioning of the body. The tumor can affect individuals irrespective of age however people between the ages of 30 and 50 have a higher incidence of getting affected with this condition.

They are slow-growing tumors however most cases of paraganglioma are non-cancerous. Approximately 90% of the condition originates from the adrenal medulla hence the disorder often referred to as extra-adrenal pheochromocytomas or paragangliomas. An individual who is hereditarily susceptible to a syndrome carries a mutated gene for the disorder. Clinical studies demonstrate that nearly 25-35 percent of the cases occur due to inheritance.

Most conditions are accompanied by symptoms such as excessive perspiration, hypertension, headaches, increased blood pressure, heart palpitation, and tremors. The latest study, according to the Pheopara Alliance, demonstrates that nearly one in 3,000 people have pheochromocytomas and/or paraganglioma.

The diagnosis would require a 24-hour urine test and blood test to measure the amount of hormone (catecholamines) in the urine and blood. Surgical resection is the only treatment available to treat the condition and more studies & research are done each year to find the cure. 3. 4.

Paragangliomas and pheochromocytomas are rare neuroendocrine tumors responsible for producing epinephrine. Also known as adrenaline, this is a hormone as well as a medication used to keep the stress under control.

This is a recurrent condition and has potential chances of revival after surgery. Your healthcare provider will suggest for lifelong follow-up care and another round of test if cancer does not return. It is often difficult to diagnose if paraganglioma is benign and malignant. When the condition becomes malignant it will grow and spread to other parts of the body.

References:

  1. “Neuroendocrine Tumors.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 25 Aug. 2020, www.mayoclinic.org/diseases-conditions/paraganglioma/cdc-20352970.
  2. Ikram, Asad. “Paraganglioma.” StatPearls [Internet]., U.S. National Library of Medicine, 30 June 2020, www.ncbi.nlm.nih.gov/books/NBK549834/.
  3. “Paragangliomas 1.” Genetic and Rare Diseases Information Center, U.S. Department of Health and Human Services, rarediseases.info.nih.gov/diseases/7324/paragangliomas-1.
  4. Patchana, Tye, et al. “Metastatic Acetabular Fracture: A Rare Disease Presentation of Recurrent Head and Neck Paraganglioma.” Cureus Journal of Medical Science, 9 Apr. 2020, www.cureus.com/articles/22966-metastatic-acetabular-fracture-a-rare-disease-presentation-of-recurrent-head-and-neck-paraganglioma.

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Team PainAssist
Team PainAssist
Written, Edited or Reviewed By: Team PainAssist, Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:September 2, 2020

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