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Review
. 2023 Feb;29(2):141-147.
doi: 10.1016/j.eprac.2022.10.002. Epub 2022 Oct 15.

Paraganglioma of the Head and Neck: A Review

Lyndsey Sandow  1 , Rajat Thawani  2 , Myung Sun Kim  3 , Michael C Heinrich  3
Affiliations
Review

Paraganglioma of the Head and Neck: A Review

Lyndsey Sandow et al. Endocr Pract. 2023 Feb.

Erratum in

Abstract

Objective: To review the epidemiology, presentation, diagnosis, and management of head and neck paragangliomas.

Methods: A literature review of english language papers with focus on most current literature.

Results: Paragangliomas (PGLs) are a group of neuroendocrine tumors that arise in the parasympathetic or sympathetic ganglia. Head and neck PGLs (HNPGLs) comprise 65% to 70% of all PGLs and account for 0.6% of all head and neck cancers. The majority of HNPGLs are benign, and 6% to 19% of all HNPGLs develop metastasis outside the tumor site and significantly compromise survival. PGLs can have a familial etiology with germline sequence variations in different susceptibility genes, with the gene encoding succinate dehydrogenase being the most common sequence variation, or they can arise from somatic sequence variations or fusion genes. Workup includes biochemical testing to rule out secretory components, although it is rare in HNPGLs. In addition, imaging modalities, such as computed tomography and magnetic resonance imaging, help in monitoring in surgical planning. Functional imaging with DOTATATE-positron emission tomography, 18F-fluorodeoxyglucose, or 18F-fluorohydroxyphenylalanine may be necessary to rule out sites of metastases. The management of HNPGLs is complex depending on pathology, location, and aggressiveness of the tumor. Treatment ranges from observation to resection to systemic treatment. Similarly, the prognosis ranges from a normal life expectancy to a 5-year survival of 11.8% in patients with distant metastasis.

Conclusion: Our review is a comprehensive summary of the incidence, mortality, pathogenesis, presentation, workup and management of HNPGLs.

Keywords: head and neck neoplasms; neuroendocrine tumors; paraganglioma.

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Figures

Fig. 1.
Fig. 1.
Computed tomography and DOTATATE-positron emission tomography in a patient with paraganglioma.
Fig. 2.
Fig. 2.
Hematoxylin and eosin staining of paraganglioma showing pink epitheloid cells with round hyperchromatic nucleus (arrow).
Fig. 3.
Fig. 3.
Algorithm summarizing the management of head and neck paraganglioma (HNPGL). CN = cranial nerve; CT = computed tomography; MRI = magnetic resonance imaging; PGL = paraganglioma; SRS = stereotactic radiosurgery.
See this image and copyright information in PMC

References

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