Management of advanced laryngeal and hypopharyngeal plexiform neurofibroma in adults

Abstract

Laryngeal neurofibromas are rare. The plexiform subtype is less common and often presents during childhood in association with neurofibromatosis type I. Because it is unencapsulated and more aggressive, plexiform neurofibroma presents a management dilemma. Imaging, particularly magnetic resonance imaging, can aid in diagnosing neurofibroma, differentiating the nonplexiform and plexiform subtypes, and planning the subsequent operative approach. The importance of conservative surgery to relieve symptoms, but preserve laryngopharyngeal function, and close follow-up are stressed. We report 2 adult cases of plexiform neurofibroma involving the larynx and hypopharynx and discuss the controversies in management.