What Leads To Paraganglioma & Can It Be Cured?

Abnormalities in the genes such as succinate dehydrogenase sub-units B, C, and mutations in the RET gene are potential factors of paraganglioma.1,2

Paraganglioma is a rare type of cancer and the treatment often depends on the location of the tumor and whether the abnormal growth is cancerous.3

Although there are no cures for metastatic paraganglioma, the treatment aims in reducing the symptoms and prolonging the survival time.4

What Leads To Paraganglioma?

It is unclear what causes the condition however clinical studies demonstrate that Paraganglioma is caused by a mutation in certain genes. This gene mutation can enhance the risk of developing Paraganglioma. Nearly 35%-40% of all PGL/PCC is caused due to inheritance as a part of familial paraganglioma syndrome.

However, multiple endocrine neoplasia type 2, von Hippel-Lindau syndrome or neurofibromatosis also tend to develop the disorder. Furthermore, a gene defect can trigger familial paraganglioma syndrome. Compared to sporadic forms, familial paragangliomas are inherited in an autosomal dominant pattern wherein just a copy of the altered gene has the potential to enhance the risk of developing cancer.

Mutations in the SDHD gene have also been associated with a malignant paraganglioma. Abnormalities in the genes such as succinate dehydrogenase sub-units B, C, and mutations in the RET gene are potential factors of paraganglioma.1,2

Can Paraganglioma Be Cured?

Paraganglioma is a rare type of cancer and the treatment often depends on the location of the tumor and whether the abnormal growth is cancerous.

The diagnosis will indicate the abnormal growth of the disorder and identify whether the condition is cancerous. Treatment aims in easing the symptoms (blood pressure) and control the spread of the cancerous cells. The treatment includes

Medications To Control Hormone Levels- The primary objective of using medications is to destroy the cancerous cells. Drugs maintain the blood pressure levels, especially a type of drug called alpha-blockers used in the combination of other drugs reduces the blood pressure and relaxes smooth muscles in the body.3

Surgery For The Removal Of Tumor- Surgery is used to completely remove the tumor cells including the ones that have spread to different parts of the human system. However, it is more critical to control the blood pressure before surgery because increased blood pressure can result in stroke during any surgery or therapy

Radiation Therapy- Paraganglioma can be cured using high-intensity radiation therapy. This procedure is often helpful in both controlling the tumor as well as improving the symptoms.

Chemotherapy- This technique destroys the cancerous cells and diminishes the ability of tumor cells to multiply and divide. When people are experiencing metastatic paraganglioma, chemotherapy is one of the most recommended options.

When the tumor is growing slowly, surgery will not be required, instead, radiation can be performed to cure the condition. Although medical studies state that there are no cures for metastatic paraganglioma, the treatment aims in reducing the symptoms and prolonging the survival time.4

Paraganglioma is a rare, benign (non-cancerous tumor) that progresses very slowly and is often related to pheochromocytoma. The origination of the tumor is from the peripheral nervous system characterized by the production of excess hormones called catecholamines.

Catecholamines are hormones secreted by the adrenal glands’ location on the anterior portion of your kidney. They act both as neurotransmitters and hormones that help the body respond to stress and fright. The hormone release is triggered during stress.

References:

  1. “Pheochromocytoma and Paraganglioma Treatment (PDQ®)–Patient Version.” National Cancer Institute, www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq.
  2. “Hereditary Paraganglioma-Pheochromocytoma – Genetics Home Reference – NIH.” U.S. National Library of Medicine, National Institutes of Health, ghr.nlm.nih.gov/condition/hereditary-paraganglioma-pheochromocytoma.
  3. Jimenez, Camilo. “Treatment for Patients With Malignant Pheochromocytomas and Paragangliomas: A Perspective From the Hallmarks of Cancer.” Frontiers, Frontiers, 11 May 2018, www.frontiersin.org/articles/10.3389/fendo.2018.00277/full.
  4. “Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Patient Information [NCI].” Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Patient Information [NCI] | Michigan Medicine, www.uofmhealth.org/health-library/ncicdr0000270724.

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