Case of spontaneous regression of carotid body tumor in a SDHD mutant: a discussion on potential mechanisms based on a review of the literature

Sebastiaan Hammer,Mark C Kruit,Frederik J Hes,Eleonora P M Van Der Kleij-Corssmit,Jeroen C Jansen

doi:10.1186/1477-7819-10-218

Sebastiaan Hammer, Mark C Kruit + Show 3 more

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https://doi.org/10.1186/1477-7819-10-218

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Abstract

BackgroundHead and neck paragangliomas are tumors associated with the parasympathetic nerve system and typically show an indolent growth pattern. Therefore a conservative management strategy is considered in selected cases.Methods and resultsWe present a case of a female patient who presented in 2003 with bilateral carotid body tumors and a tympanic tumor, associated with a mutation in the succinate dehydrogenase -sub-unit-D (SDHD). She was operated on the right carotid body tumor and the tympanic tumor. Thereafter the follow-up was performed with MR examinations at 2-year intervals. After an initial stable phase, over the last 3 years a spontaneous near-total regression of the contralateral carotid body tumor was observed, with only subtle rest-abnormalities visible in 2011.ConclusionsThe present case underlines the indolent growth pattern of head and neck paragangliomas and for the first time describes a rare manifestation of spontaneous regression of a carotid body tumor. The literature was reviewed to discuss this phenomenon.

Highlights

Head and neck paragangliomas are tumors associated with the parasympathetic nerve system and typically show an indolent growth pattern
The present case underlines the indolent growth pattern of head and neck paragangliomas and for the first time describes a rare manifestation of spontaneous regression of a carotid body tumor
Head and neck paragangliomas (HNPGL) are usually benign, slow-growing tumors associated with the parasympathetic nerve system

Summary

Introduction

Head and neck paragangliomas are tumors associated with the parasympathetic nerve system and typically show an indolent growth pattern. A conservative management strategy is considered in selected cases. Head and neck paragangliomas (HNPGL) are usually benign, slow-growing tumors associated with the parasympathetic nerve system. A paraganglioma presents as a non-tender mass or as a cause of lower cranial nerve palsy due to local compression. The first symptom of paragangliomas is usually pulsating tinnitus, due to the hypervascular nature of the tumor. Treatment considerations include the nature of the tumor (malignant or benign), the location, vasculature encasement, the extent, and growth rate [6,7]. Because of the slow growth rate and potential treatment-related injury to the neighboring vessels and nerves, a conservative management strategy (wait-and-scan policy) should be considered [6]

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