What Are The Ways To Prevent Paraganglioma & Does It Reoccur?

Clinical studies state that there is no known way to prevent paraganglioma nevertheless genetic testing is often helpful in early diagnosis. 1. 2.

People with paraganglioma need life-long follow-up care because it can recur anytime after initial treatment. 3.

Tumors are recurrent in nature and distant metastases can occur and therefore vigilance is needed in these patients. 4.

What Are The Ways To Prevent Paraganglioma?

Clinical studies state that there is no known way to prevent paraganglioma nevertheless genetic testing is often helpful in early diagnosis. Most cases of cancer are hereditary therefore when children have known genetic disorders with Pheochromocytomas/Paragangliomas should be tested for the presence of the syndrome. Early diagnosis of the disorder helps prevent other associating cancers.

Clinical findings primarily aim towards a specific genetic disorder such as (Multiple endocrine neoplasia type) MEN1, MEN2, (Von Hippel-Lindau syndrome) VHL disease, (Neurofibromatosis type 1) NF1 and the objective of the testing is to identify the abnormalities related to the specific disorder. Children who have increased risk for MEN2 should have annual follow-up care to check for the presence of tumors or analyze if they are developing any other signs and symptoms of paraganglioma.

Screening the RET gene mutation at an early age can prevent the occurrence of medullary carcinoma and enhance better diagnosis and therapy. However, in rare cases where there are no obvious symptoms, the children should be tested on the SDHB mutation on the initial level and SDHD mutation in the next level to check for the reversal of the condition.

Endocrine society has provided a complete set of guidelines on all the procedures for genetic testing. 1. 2.

Does Paraganglioma Reoccur?

People with paraganglioma need life-long follow-up care because it can recur anytime after initial treatment. The evidence available on the nature of the paraganglioma and its associated conditions is diverse and contradictory.

A medical study was conducted by a multicenter study in Piedmont, Italy to determine the causing factor of recurrence in people who were experiencing paraganglioma. These patients were subjected to radical surgery and data was obtained for two hundred and forty-two patients between the years 1990 and 2016. 3.

About 17% of the patients had disease recurrence but showed they acquired the condition due to genetic mutation. These cases were identified with larger tumor size and an increased risk of recurrence rate of pheochromocytoma and paraganglioma. The study concluded that the recurrence of pheochromocytoma and sympathetic paraganglioma was more frequent in the younger children who reported a family history of chromaffin tissue neoplasms, and genetic mutations.

Tumors are recurrent in nature and distant metastases can occur and therefore vigilance is needed in these patients. Regular follow-up care and biochemical tests are essential after surgery because one-tenth of the population was frequently diagnosed with paraganglioma recurrence. 4.

Paragangliomas are tumors that originate in the adrenal glands in the back of the upper abdomen, an accumulation of cells (chromaffin cells) that is often related to the vascular and neuronal adventitia. These cells produce a hormone that is essential for the proper functioning of the body.

Almost half of the paragangliomas occur in the adrenal medulla and a small portion (10%) originates outside the area. The condition is typically diagnosed in the head/neck, chest, and abdomen, and the symptoms involve headache, excessive sweating, tremors, irregular heartbeat, and high blood pressure.

References:

  1. “Pheochromocytoma and Paraganglioma – Latest Research.” Cancer.Net, 20 Nov. 2019, www.cancer.net/cancer-types/pheochromocytoma-and-paraganglioma/latest-research.
  2. Erickson, Dana, et al. “Benign Paragangliomas: Clinical Presentation and Treatment Outcomes in 236 Patients.” OUP Academic, Oxford University Press, 1 Nov. 2001, academic.oup.com/jcem/article/86/11/5210/2849297.
  3. Johnston, Philip C, et al. “Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention.” The Ulster Medical Journal, The Ulster Medical Society, May 2015, www.ncbi.nlm.nih.gov/pmc/articles/PMC4488930/.
  4. Parasiliti-Caprino, Mirko, et al. “Predictors of Recurrence of Pheochromocytoma and Paraganglioma: a Multicenter Study in Piedmont, Italy.” Nature News, Nature Publishing Group, 4 Oct. 2019, www.nature.com/articles/s41440-019-0339-y.

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