Glomus Vagale of the Parapharyngeal Space: Working toward a Simplified Treatment Paradigm

Background Parapharyngeal space (PPS) glomus vagale (GV) are exceptionally rare, benign, post-styloid tumors that arise from neoplastic non-chromaffin paraganglion cells of the vagus nerve. As compared with their more common counterparts, GV represent less than 3% of all head-and-neck paragangliomas, with fewer still arising in the PPS. Correspondingly, we sought to describe their natural history and optimal management in a large, single-institution series.

Methods Retrospective institutional PPS database, assembled via pathology, anatomic site, and diagnosis codes, was queried for GV, 1965 to 2015, with supplemental secondary retrospective chart review conducted as required.

Results Thirty-three PPS GV patients were identified, of whom 24 (73%) were female (Table 1; median age 46, range: 14–75). Median tumor size was 4.0 cm (range: 0.6–7.0). Most presented with neck mass (n = 23, 70%), causing dysphagia or hoarseness in one-in-three (n = 9, 27%). Headaches were common (n = 10, 30%), but other signs of catecholamine excess were infrequent, including syncope (n = 3, 9%) and hypertension (n = 2, 6%). Catecholamine secretion status was tested in 21 (64%), of whom three were positive (14%). Preoperative angiography/embolization was infrequent (n = 3, 9%). Surgical approach was transparotid cervical in 17 (52%), transcervical in 15 (45%), and transoral in 1 (3%). Key neurovascular involvement included the vagus nerve (n = 33, 100%), hypoglossal nerve (n = 16, 49%), and internal carotid artery (ICA; n = 12, 36%). Postoperative outcomes included universal vocal cord paralysis, and frequent swallowing dysfunction, with acute symptomatic dysphagia in 14 (42%), of whom 5 (15%) had persistent symptoms at last follow-up (no PEG tubes required). Other complications were rare, and included hypoglossal weakness in 9 (27%), shoulder weakness in 3 (9%), Horner's syndrome in 2 (6%), aspiration pneumonia in 2 (6%), and deep vein thrombosis without pulmonary embolism in 1 (3%). As of last follow-up (median 47 months; range: 0–504), there were no mortalities attributable to primary disease or treatment complication; durable local control was confirmed by imaging in 29 (88%).

Conclusion GV are rare PPS tumors that require nuanced management. In the present series, catecholamine screening was frequently performed, with positive results identified in at least 14% of patients, supporting the continuation and expansion of this practice. Preoperative angiography was infrequently performed, suggesting that excellent outcomes may be achieved with or without embolization, if applied to appropriately selected patients. Further study is required to establish evidence-based workup and treatment protocols; however, our preliminary results indicate that these patients may benefit from simplified assessments focused on surgical planning with a multidisciplinary otolaryngology—head and neck surgery and neurosurgery team.

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