Solitary Isolated Oropharyngeal Neurofibroma Presenting With Dysphagia in the Setting of Von Recklinghausen's Disease.

Abstract

A 24-year-old man with von Recklinghausen's disease presented with complaints of difficulty in swallowing for 6 months and change of voice for 3 months. He also had recent-onset difficulty in breathing. Telelaryngoscopy and subsequent contrast-enhanced computed tomography scan revealed a well-defined, smooth submucosal mass in the oropharynx (attached to the posterior pharyngeal wall, superior to the level of left aryepiglottic fold), obscuring the ipsilateral pyriform fossa, and nearly blocking the pharyngeal lumen. The mass was removed with endoscopic coblation-assisted laryngeal surgery, and subsequent histopathology revealed it to be neurofibroma. Neurofibromas are rare neoplasms to be encountered in the oropharynx. However, in the setting of von Recklinghausen's disease (neurofibromatosis type 1), one or more well-demarcated, submucosal nodular lesions in the upper aerodigestive tract may be considered as neurofibromas, and workup and treatment should be directed accordingly based on this clinical presumption. Endoscopic coblation during laryngeal surgery can effectively be used as a surgical tool to excise such lesions. It provides a relatively bloodless field compared to the conventional cold steel excision, and reduces the risk of complications at surgery and during the follow-up period. This clinical record illustrates the presentation and management of a solitary, isolated oropharyngeal neurofibroma in a man suffering from von Recklinghausen's disease. It further emphasizes the role of endoscopic coblation-assisted laryngeal surgery in this setup, and the need to maintain a low threshold of suspicion in having a provisional clinical diagnosis of such lesions.