Solitary plexiform neurofibroma: An unusual clinical presentation on the lip

Abstract

Neurofibromas (NF) are seen either as a solitary lesion or as part of the generalized syndrome of NF (NF-1, also known as von Recklinghausen disease of the skin). In plexiform neurofibroma (PN), there is a proliferation of Schwann cells from the inner aspect of the nerve sheath, thereby resulting in an irregularly thickened, distorted, and tortuous structure. Oral involvement by a solitary and peripheral PN in patients with no other signs of NF is rarely seen. It is reported that only 4-7% of patients affected by NF display oral manifestations. A solitary PN in a patient with no other symptoms is a diagnostic challenge, more so when the location of the lesion is one of the rarest sites.