Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.Keywords Paraganglioma Á Carotid body tumor Á Malignant paraganglioma
HistoryA 25 year-old female reported to the emergency department for evaluation of a severe headache. An avid singer, she incidentally complained of difficulty with vocal range and stamina over several months. Family history was negative for head and neck cancer. Physical exam revealed fullness with mild generalized erythema of both the left posterior nasopharynx and the oropharynx. On palpation of the left oropharynx there was no discrete mass, however, the patient endorsed mild tenderness on palpation. Flexible fiberoptic laryngoscopic exam revealed normal bilateral true vocal fold motion and appearance. No cervical lymphadenopathy was appreciated and cranial nerves II-XII were intact.
Radiographic FeaturesComputed tomography (CT) identified a 6 9 4 9 3 cm left post-styloid parapharyngeal space avidly enhancing mass (Fig. 1). Magnetic resonance imaging (MRI) confirmed a mass within the left parapharyngeal space that was isointense on T1, slightly hyperintense on T2, and showed multiple flow voids with gadolinium enhancement. The mass was posterior to the carotid bifurcation and anterolateral to the internal jugular vein. Radiographic findings were highly suspicious for a vagal paraganglioma or nerve sheath tumor. Urine catecholamines, vanillylmandelic acid (VMA) panel and thyroid function studies were all within normal limits. Succinyl dehydrogenase (SDH) gene testing was negative.Disclaimer: The opinions and assertions expressed herein are those of the authors and are not to be construed as official or representing the views of the Department of the Navy or the Department of Defense. I certify that all individuals who qualify as authors have been listed; each has participated in the conception and design of this work, the writing of the document, and the approval of the submission of this version; that the document represents valid work; that if we used information derived from another source, we obtained all necessary approvals to use it and made appropriate acknowledgements in the document; and that each takes public responsibility for it. We are military service members.