Is Paraganglioma A Neuroendocrine Tumor & Is There A Surgery For It?

A paraganglioma is a rare neuroendocrine neoplasm that occurs near certain blood vessels or nerves outside the adrenal glands.1,2

Most cases of paraganglioma are non-cancerous however untreated condition becomes malignant, grow, and spread to the distant parts of the body requiring surgery to remove the tumor.3

Surgery is the best choice of treatment to remove the tumor if feasible and also reduce its size when it has spread to different parts of the body.4

Is Paraganglioma A Neuroendocrine Tumor?

A neuroendocrine tumor can develop in any part of the body but the rectum, pancreas, and the digestive tract are most affected with the condition. They can be either cancerous or non-cancerous depending on the location, size, and stage of the tumor. They are a relatively rare form of cancer that forms near certain blood vessels and nerves outside of the adrenal glands.

A tumor begins when the DNA of healthy cells is damaged, affecting the cells to alter and develop out of control at various body sites including the head, neck, thorax, and abdomen but can infect nearby parts of the body, become tumorous (cancerous) and propagate distantly (metastasize). Most paragangliomas show no symptoms and are painless condition.

Almost half of the cases of paraganglioma produces abnormal cells known as catecholamines, a hormone that performs the flight and fright function. When there is excess secretion of this hormone, it may induce high blood pressure, profuse sweating, headache, and tremors.1,2

Is There A Surgery For Paraganglioma?

Most cases of paraganglioma are non-cancerous however untreated condition becomes malignant, grow, and spread to the distant parts of the body requiring surgery to remove the tumor. Before the surgery, there are tests and procedures to diagnose the condition. This includes:
Physical exam and history to check signs and health that seems unusual. This procedure will also check the patient’s health history and treatments would be based on that.

24-hour urine test to measure the number of catecholamines present in the urine. When the hormone level increases it may be a sign of pheochromocytoma.

Imaging studies like computed tomography, computerized tomography, or computerized axial tomography scan to makes a series of detailed pictures of areas inside the body 3.

There are different types of treatment for patients with this condition. Surgery is the best choice of treatment to remove the tumor if feasible and also reduce its size when it has spread to different parts of the body.

Surgery involves the removal of one or more adrenal glands and the procedure is called adrenalectomy. During the surgery, the lymph nodes and tissues inside the adrenal glands would be examined to determine the spread and if it has metastasized, these would also require removal of these tissues.

When both the adrenal glands are removed, lifelong hormone therapy would be required to replace the lost hormones.4

A paraganglioma is an extra-adrenal rare tumor that develops from the parasympathetic nervous system forming ganglia. The incidence of this condition is approximately 2-8 people per million every year. They are usually benign and do not produce many symptoms and are rarely noticed in the central nervous system.

A major percentage of central nervous system paragangliomas manifest as carotid and slow-growing benign tumors. Surgical treatment provided the best possible outcomes however long-term follow-up care was important to improve success in treatments.

References:

  1. From the *Department of Nuclear Medicine & PET. “Paraganglioma and Pancreatic Neuroendocrine Tumor with Rare … : Clinical Nuclear Medicine.” LWW, journals.lww.com/nuclearmed/Abstract/2015/03000/Paraganglioma_and_Pancreatic_Neuroendocrine_Tumor.18.aspx.
  2. Allan, Ronny. “Neuroendocrine Tumours: a Spotlight on Pheochromocytoma and Paraganglioma.” Ronny Allan – Living with Neuroendocrine Cancer, 27 July 2020, ronnyallan.net/2016/12/14/neuroendocrine-tumours-a-spotlight-on-pheochromocytomas-and-paragangliomas/.
  3. “Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI].” Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente, healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/
  4. García, Maria Isabel Del Olmo. “Surgical and Pharmacological Management of Functioning Pheochromocytoma and Paraganglioma.”
  5. Paraganglioma: A Multidisciplinary Approach [Internet]., U.S. National Library of Medicine, 2 July 2019, www.ncbi.nlm.nih.gov/books/NBK543229/.

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