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 Table of Contents  
Year : 2020  |  Volume : 53  |  Issue : 3  |  Page : 121-122

Head-and-neck paragangliomas

Department of Neurology; Department of Medicine, Federal University of Santa Maria, Santa Maria, Rio Grande do Sul, Brazil

Date of Submission29-Nov-2019
Date of Acceptance20-Feb-2020
Date of Web Publication30-May-2020

Correspondence Address:
Dr. Jamir Pitton Rissardo
Rua Roraima, Santa Maria, Rio Grande do Sul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_105_19

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How to cite this article:
Rissardo JP, Caprara AL. Head-and-neck paragangliomas. Formos J Surg 2020;53:121-2

How to cite this URL:
Rissardo JP, Caprara AL. Head-and-neck paragangliomas. Formos J Surg [serial online] 2020 [cited 2021 Aug 4];53:121-2. Available from: https://www.e-fjs.org/text.asp?2020/53/3/121/285397

Dear Editor,

We read the article entitled, “Multiple paragangliomas involving the carotid body and vagal region” on the esteemed “Formosan Journal of Surgery” with great interest. Bakshi et al. reported a case of an adult male with a swelling in the left upper part of the neck. Their report is the second case of multiple paragangliomas affecting the carotid body and vagal region.[1]

A systematic review of the genetic analysis of paragangliomas from head and neck showed that 10 out of 15 genes only when mutated can turn the individual susceptible to the development of these tumors. In this way, they discussed the influence of the main genetic drivers on the somatic molecular phenotype showed by DNA methylation and microRNA profiling. They stated that assuming a case of sporadic without doing a careful clinical observation could lead to a high number of misdiagnosis. Some of the genetic mutations associated with paragangliomas can skip generations or have variable penetrance, so in every case where these tumors are encountered a pedigree description should be done.[2]

An interesting fact is about how much a metastasis in the regional lymph nodes can affect the survival rate. Javidiparsijani et al. in their retrospectively study on 65 patient charts found that about 50% of the patients had regional lymph node biopsy positive. After they evaluate the follow-up of these individuals, no evidence of local recurrence or distant metastasis in the patients with positive lymph nodes was found within 6–11 years of follow-up. Therefore, in most cases where there is metastasis to local lymph nodes, an indolent course is observed however, if a positive lymph node is noted in the sclerosing variant, the prognosis is worst.[3]

Another recent study by Singh et al. has evaluated the epidemiological profile, clinicopathological correlation, and management of head-and-neck paragangliomas in 54 Indian individuals. Their study revealed that the site with the largest and most common paragangliomas is the carotid body in the head-and-neck region.[4]

We would like to provide the mnemonic “CAROT ↑ DS” to help remember the main facts about the head-and-neck paragangliomas: Carotid body tumors are the largest and most common paragangliomas of the head and neck. Autonomic neoplasm origin can be classified based on the lesion site. RET, SDHB, SDHD, and VHL germline mutations are associated with familial cases. Occurrence in the head and neck is about 3% of all paragangliomas. Tumor resection is one of the management options but does not improve life expectancy. : More upper (↑) in the body is more probable the P(↑) arasympathetic origin. Death and morbidity are more common with glomus jugulotympanicum tumors. Sclerosing variant of paraganglioma has a more aggressive histological behavior, especially when lymph nodes are positive.[1],[2],[3],[4],[5]

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  References Top

Bakshi J, Goyal AK, Vir D, Panda NK. Multiple paragangliomas involving carotid body and vagal region. Formos J Surg 2019;52:143.  Back to cited text no. 1
  [Full text]  
Guha A, Musil Z, Vicha A, Zelinka T, Pacak K, Astl J, et al. A systematic review on the genetic analysis of paragangliomas: Primarily focused on head and neck paragangliomas. Neoplasma 2019;66:671-80.  Back to cited text no. 2
Javidiparsijani S, Brickman A, Lin DM, Rohra P, Ghai R, Bitterman P, et al. Is Regional Lymph Node Metastasis of Head and Neck Paraganglioma a Sign of Aggressive Clinical Behavior: A Clinical/Pathologic Review [published online ahead of print, 2019 Sep 29]. Ear Nose Throat J 2019;145561319863373. doi:10.1177/0145561319863373.  Back to cited text no. 3
Singh S, Madan R, Singh MK, Thakar A, Sharma SC. Head-and-neck paragangliomas: An overview of 54 cases operated at a tertiary care center. South Asian J Cancer 2019;8:237-40.  Back to cited text no. 4
[PUBMED]  [Full text]  
Agarwal G, Rajan S, Valiveru RC, Tulsyan S, Agrawal V, Mittal B, et al. Genetic profile of Indian pheochromocytoma and paraganglioma patients-A single institutional study. Indian J Endocrinol Metab 2019;23:486-90.  Back to cited text no. 5


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