Solitary Plexiform Neurofibroma of the Gingiva: Unique Presentation in the Oral Cavity

Abstract

Neurofibromas are benign tumours originating from the nerve sheath. Amongst the histological variants, plexiform types are considered exclusive. These are poorly circumscribed, locally invasive and may exhibit sarcomatous potential. Plexiform neurofibromas are key features of Neurofibromatosis - 1 and their solitary intra-oral presentation is uncommon. The following case report describes a unique case of an isolated solitary plexiform neurofibroma of the maxillary anterior gingival region in a middle aged female patient.

Solitary Encapsulated Neurofibroma Not Associated with Neurofibromatosis-1 Affecting Tongue in a 73-Year-Old Female

“…One such neoplasm occurring in the tongue is neurofibroma which is a benign peripheral nerve sheath tumor arising due to proliferation of Schwann cells, perineurial cells, and endoneurial fibroblasts [ 1 – 5 ]. It can present either as a localized lesion or as part of generalized syndrome known as Neurofibromatosis type 1 (NF-1) or Von Recklinghausen's disease or rarely with Type III Multiple Endocrine Neoplasia (MEN-III) Syndrome [ 1 – 4 , 6 ]. Based on clinical presentation, it is classified as solitary or multiple [ 1 – 6 ].…”

“…It is rarely seen in the oral cavity [ 1 , 3 ]. Intraorally, only 6.5% of cases of solitary neurofibromas have been reported which are not associated with NF-1 [ 4 ]. It can affect a wide age group from 10 months to 70 years, though it is commonly found in 3rd decade [ 3 , 6 ].…”

“…Histologically, solitary neurofibroma is usually unencapsulated and consists of interlacing bundles of spindle shaped cells with wavy nuclei in fibrous or myxomatous stroma [ 1 , 2 , 5 , 9 ]. Numerous mast cells and lymphocytes are usually scattered in the connective tissue [ 1 , 2 , 4 – 6 , 8 – 10 ].…”

“…Immunohistochemically, the tumor cells show positive reactivity for S-100 protein (in 85% to 100% of cases), neuron specific enolase, and vimentin [ 1 , 2 , 7 , 8 ]. Complete surgical excision is the treatment of choice [ 2 – 4 , 9 , 10 ]. The rate of malignant transformation is about 3% to 15%, especially when associated with NF-1 [ 3 , 4 , 10 ].…”

“…Complete surgical excision is the treatment of choice [ 2 – 4 , 9 , 10 ]. The rate of malignant transformation is about 3% to 15%, especially when associated with NF-1 [ 3 , 4 , 10 ].…”