Diagnosis and Management of Cervical Sympathetic Chain Paraganglioma

Abstract

A 46 year old man presented with a four week history of swelling in the back of his throat and slowly enlarging right sided neck mass. He had onset of anisocoria and mild right-sided ptosis three years prior, and had been diagnosed with Horner's syndrome at that time. He developed constant raspy hoarseness one year ago. He denied dysphagia, dyspnea, palpitations, hypertension, otalgia, weight loss, and night sweats. He had no significant medical history, and no family history of paraganglioma.Physical examination revealed anisocoria with constricted right pupil along with right eyelid ptosis (Figure 3). The right level II neck had a hard, immobile 5 cm mass. The right posterior and lateral oropharynx had a visible submucosal fullness (Figure 4). On flexible nasopharyngoscopy, this fullness extended a total of 5 cm along the right lateral pharyngeal wall.Contrast CT showed an enhancing mass in the right parapharyngeal space measuring 5.2 x 3.1 cm that originated near the carotid bifurcation. The external and internal carotid artery (ECA and ICA) were mildly splayed at their bifurcation and superiorly the mass displaces both the vessels anterolaterally. Contrasted MRI reveals similar findings with flow voids on T2 imaging ( Figure 5).

Paragangliomas of the head and neck: a contemporary review

“…Common presenting signs include ipsilateral Horner syndrome (partial ptosis, miosis, and anhidrosis) and oropharyngeal fullness. Sympathetic chain PGLs more commonly secrete catecholamines compared to other PGLs, though all HNPGLs may potentially possess this feature ( Seth et al 2014 ). Other exceedingly rare HNPGL subsites include laryngeal PGLs, arising from the supraglottic or subglottic larynx and presenting with dysphonia or airway obstruction ( Myssiorek et al 2004 ), and parotid gland PGLs ( Vora et al 2012 ).…”