Primary paraganglioma located between the thyroid gland and the left common carotid artery: A case report

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Abstract

Head and neck paraganglioma is a rare and predominantly asymptomatic tumor. In the present study, an extremely rare case of asymptomatic paraganglioma located between the left common carotid artery and the left thyroid is described. The clinical presentation, cytomorphology and the immunohistochemical characteristics for the diagnosis of head and neck paraganglioma are described. To the best of our knowledge, only two cases of paraganglioma located between the left common carotid artery and the left thyroid have previously been reported.

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Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma.

B E Baysal, R Ferrell, J Willett-Brozick … (2000)

Hereditary paraganglioma (PGL) is characterized by the development of benign, vascularized tumors in the head and neck. The most common tumor site is the carotid body (CB), a chemoreceptive organ that senses oxygen levels in the blood. Analysis of families carrying the PGL1 gene, described here, revealed germ line mutations in the SDHD gene on chromosome 11q23. SDHD encodes a mitochondrial respiratory chain protein-the small subunit of cytochrome b in succinate-ubiquinone oxidoreductase (cybS). In contrast to expectations based on the inheritance pattern of PGL, the SDHD gene showed no evidence of imprinting. These findings indicate that mitochondria play an important role in the pathogenesis of certain tumors and that cybS plays a role in normal CB physiology.

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Mutations in SDHC cause autosomal dominant paraganglioma, type 3.

S. Niemann, U Müller (2000)

Nonchromaffin paragangliomas (PGLs) are usually benign, neural-crest-derived, slow-growing tumours of parasympathetic ganglia. Between 10% and 50% of cases are familial and are transmitted as autosomal dominant traits with incomplete and age-dependent penetrance.

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Head and neck paragangliomas: clinical and molecular genetic classification

Christian Offergeld, Christoph Brase, Svetlana Yaremchuk … (2012)

Head and neck paragangliomas are tumors arising from specialized neural crest cells. Prominent locations are the carotid body along with the vagal, jugular, and tympanic glomus. Head and neck paragangliomas are slowly growing tumors, with some carotid body tumors being reported to exist for many years as a painless lateral mass on the neck. Symptoms depend on the specific locations. In contrast to paraganglial tumors of the adrenals, abdomen and thorax, head and neck paragangliomas seldom release catecholamines and are hence rarely vasoactive. Petrous bone, jugular, and tympanic head and neck paragangliomas may cause hearing loss. The internationally accepted clinical classifications for carotid body tumors are based on the Shamblin Class I–III stages, which correspond to postoperative permanent side effects. For petrous-bone paragangliomas in the head and neck, the Fisch classification is used. Regarding the molecular genetics, head and neck paragangliomas have been associated with nine susceptibility genes: NF1, RET, VHL, SDHA, SDHB, SDHC, SDHD, SDHAF2 (SDH5), and TMEM127. Hereditary HNPs are mostly caused by mutations of the SDHD gene, but SDHB and SDHC mutations are not uncommon in such patients. Head and neck paragangliomas are rarely associated with mutations of VHL, RET, or NF1. The research on SDHA, SDHAF2 and TMEM127 is ongoing. Multiple head and neck paragangliomas are common in patients with SDHD mutations, while malignant head and neck paraganglioma is mostly seen in patients with SDHB mutations. The treatment of choice is surgical resection. Good postoperative results can be expected in carotid body tumors of Shamblin Class I and II, whereas operations on other carotid body tumors and other head and neck paragangliomas frequently result in deficits of the cranial nerves adjacent to the tumors. Slow growth and the tendency of hereditary head and neck paragangliomas to be multifocal may justify less aggressive treatment strategies.

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Author and article information

Journal

Journal ID (nlm-ta): Oncol Lett

Journal ID (iso-abbrev): Oncol Lett

Journal ID (publisher-id): OL

Title: Oncology Letters

Publisher: D.A. Spandidos

ISSN (Print): 1792-1074

ISSN (Electronic): 1792-1082

Publication date (Print): November 2014

Publication date (Electronic): 11 August 2014

Publication date PMC-release: 11 August 2014

Volume: 8

Issue: 5

Pages: 1925-1928

Affiliations

[1 ]Department of Endocrinology and Metabolism, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing, Zhejiang 312000, P.R. China

[2 ]Department of Breast and Thyroid Surgery, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing, Zhejiang 312000, P.R. China

[3 ]Department of Pathology, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, Shaoxing, Zhejiang 312000, P.R. China

Author notes

Correspondence to: Dr Feng Xu, Department of Breast and Thyroid Surgery, Shaoxing People’s Hospital, Shaoxing Hospital of Zhejiang University, 568 Zhongxing Road, Shaoxing, Zhejiang 312000, P.R. China, E-mail: xufengshaoxing@ 123456163.com

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Publisher ID: ol-08-05-1925

DOI: 10.3892/ol.2014.2432

PMC ID: 4186629

SO-VID: 0af8dbb6-7f23-4380-8823-f4b3bf3e4106

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This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited.

Primary paraganglioma located between the thyroid gland and the left common carotid artery: A case report

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Hematology and Oncology Discovery

Most cited references 13

Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma.

Mutations in SDHC cause autosomal dominant paraganglioma, type 3.

Head and neck paragangliomas: clinical and molecular genetic classification

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