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Case report
A 36-year-old man presented to the clinic with a 3-month history of an enlarging right-sided neck mass. He had some associated pain in the region but denied any dysphonia, dysphagia, flushing, palpitations, diarrhea, or previous syncopal episodes. He denied a history of tobacco use, chronic alcohol use, or a family history of paragangliomas. Physical examination demonstrated a right-sided level II neck mass measuring approximately 5 cm in anterior-posterior dimension that was tender to touch. Palpation of the neck did not reveal any additional lymphadenopathy. All cranial nerves were functional with particular attention to cranial nerve X: he had symmetric palatal elevation and normal laryngeal function on fiberoptic nasopharyngolaryngoscopy. The mucosal surface of the entire upper aerodigestive tract was unremarkable.
Magnetic resonance imaging (MRI)/magnetic resonance angiography demonstrated a 3.9 × 3.6 × 5.0-cm multilobulated parapharyngeal mass displacing the carotid artery anteromedially and the jugular vein posteromedially with slight compression ( Fig. 1 ), most consistent with a glomus vagale tumor. When compared with a previous MRI performed 3 months earlier, there was interval growth of the mass of approximately 0.5 cm. A whole-body octreotide scan failed to show any additional areas of uptake. The patient was presented at our multidisciplinary tumor board. After extensive discussion of the options in treating vagal paragangliomas along with the functional sequela of surgical resection and the reconstructive options, the patient decided against pursuing a radiation oncology consult but rather opted for a surgical resection. A vascular surgery consult was obtained, and the patient underwent preoperative embolization of the right ascending pharyngeal artery with an Onyx injection (ethylene vinyl alcohol copolymer) less than 24 hours before the procedure.
Surgical resection involved a right parapharyngeal space dissection along with a selective neck dissection (levels 2–3) via a modified Blair incision with a cervical extension. External jugular nodes were sent for frozen pathology and were found to be negative. Cranial nerves IX, XI, and XII and the sympathetic chain were identified and preserved, with X being sacrificed. The ansa hypoglossal nerve was significantly attenuated precluding its use for purposes of reinnervation. The stylohyoid and digastric muscles were divided to provide access to the proximal portion of the tumor at the level of the skull base. The tumor was removed en bloc without complication, requiring sacrifice of the superior laryngeal nerve (SLN).
Reconstitution of the anticipated defects involved the following: a left ansa cervicalis to right recurrent laryngeal nerve (RLN) neuroplasty; a right greater auricular nerve to right SLN neuroplasty, and a free nerve interposition graft from the left cricothyroid muscle to the right cricothyroid muscle ( Fig. 2 ). The contralateral ansa cervicalis nerve was used because of the attenuation found along the ipsilateral nerve, and a cut branch of the vagus that was found to be histologically normal on frozen section was used for the interposition graft.
Postoperatively, the patient had minimal issues with deglutition, requiring a feeding tube for 6 days. He did have some symptoms of first bite syndrome that slowly resolved on subsequent postoperative visits. Seven months after the operation, an examination by speech pathology demonstrated his vocal quality to be within normal limits, a pitch range of about 1½ octaves, and remarkably good loudness. He had some paresthesias of his ear lobule every time he swallowed, but no dysphagia. On videostroboscopic examination ( Fig. 3 ), he had normal glottic closure throughout the pitch range with excellent amplitude. With regards to the right vocal fold, he had good tone with a straight edge that was immobile and close to the midline. One year postoperatively, he continued to show good glottic closure and remains without evidence of recurrence both radiographically and clinically.
