Abstract

IntroductionPlexiform neurofibroma of the larynx is a rare disease. In this report, we present a plexiform neurofibroma in the arytenoids and neurofibroma in the parapharynx detected coincidently.Case presentationA 56-year-old Asian woman presented with respiratory distress and episodes of apnea at night. A solitary mass from the left arytenoids was found to be nearly obstructing the airway and causing the sleep apnea. There was also a parapharynx mass protruding into the pharynx. The parapharynx tumor was removed with the lateral incision approach, and the arytenoid tumor was removed with a transoral carbon dioxide laser. The pathologic diagnosis was plexiform neurofibroma for the arytenoid mass and neurofibroma for the parapharynx mass.ConclusionWe have reported an extremely rare case of plexiform neurofibroma in the arytenoids and neurofibroma in the parapharynx. This entity may be considered in the differential diagnosis of all laryngeal and parapharynx masses.

Highlights

  • Plexiform neurofibroma of the larynx is a rare disease

  • This entity may be considered in the differential diagnosis of all laryngeal and parapharynx masses

  • The histological stain was positive for S-100, and an examination revealed soft tissue with multiple nerve fascicles, some of which were enlarged with edematous endoneurium with intermixed collagen and bipolar cells consistent with a diagnosis of plexiform neurofibroma; the arytenoid mass was confirmed as a simple neurofibroma (Figure 3)

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Summary

Introduction

Known as von Recklinghausen disease (VRD), affects Schwann cells. The upper parapharynx wall of the left vocal cord was protruding toward the pharyngeal cavity (Figure 1). Computed tomography (CT) revealed a non-enhancing mass arising from the left side of the arytenoid cartilage and from the parapharynx and extending to the areas of the carotid artery (Figure 2). We used the lateral pharyngotomy approach for the removal of the parapharynx tumor, and removed the arytenoid tumor using a carbon dioxide (CO2) laser. The histological stain was positive for S-100, and an examination revealed soft tissue with multiple nerve fascicles, some of which were enlarged with edematous endoneurium with intermixed collagen and bipolar cells consistent with a diagnosis of plexiform neurofibroma; the arytenoid mass was confirmed as a simple neurofibroma (Figure 3). The patient complained of a voice disorder postoperatively, and left vocal cord palsy was present on physical examination. Two years postoperatively, the patient has had neither respiratory nor feeding difficulties, and her voice is nearly normal (Figure 4)

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