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Review
. 2021 Nov 26;7(1):93-107.
doi: 10.1002/lio2.706. eCollection 2022 Feb.

Contemporary management of paragangliomas of the head and neck

Eoin F Cleere  1   2 , Julie Martin-Grace  2   3 , Adrien Gendre  1   2 , Mark Sherlock  2   3 , James P O'Neill  1   2
Affiliations
Review

Contemporary management of paragangliomas of the head and neck

Eoin F Cleere et al. Laryngoscope Investig Otolaryngol. .

Abstract

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors typically arising from nonsecretory head and neck parasympathetic ganglia. Historically thought of as aggressive tumors that warranted equally aggressive surgical intervention, evidence has emerged demonstrating that the vast majority of HNPGLs are slow growing and indolent. It is also now recognized that a large proportion of HNPGLs are hereditary with succinate dehydrogenase gene mutations typically implicated. These recent advances have led to significant changes in the way in which clinicians investigate and treat HNPGLs with most now opting for more conservative treatment strategies. However, a proportion of patients present with more aggressive disease and still require nonconservative treatment strategies. Recent studies have sought to determine in which groups of patients the morbidity associated with treatment is justified. We summarize the recent advances in the understanding and management of these tumors and we provide our recommendations regarding the management of HNPGLs.

Keywords: carotid‐body tumor; glomus tumor; neuroendocrine tumors; paraganglioma; succinate dehydrogenase (SDH).

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Investigation of a suspected HNPGL. CBT, carotid body tumor; CT, computed tomography; FDG‐18, fluorodeoxyglucose‐18; Ga‐68, Gallium‐68; HNPGL, head and neck paragangliomas; MDT, multidisciplinary team meeting; MRI, magnetic resonance imaging; non‐CBT, non‐carotid body tumor; PET, Positron emission tomography; SDH, succinate dehydrogenase
FIGURE 2
FIGURE 2
Management of isolated carotid body tumor. CN, cranial nerve; MDT, multidisciplinary team; SDH, succinate dehydrogenase;
FIGURE 3
FIGURE 3
Management of isolated non‐carotid body tumor. CN, cranial nerve; HNPGL, head and neck paraganglioma; JTPGL, Jugulo‐tympanic paraganglioma; MDT, multidisciplinary team; non‐CBT, non‐carotid body tumor; SDH, succinate dehydrogenase; VPGL, vagal paraganglioma
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