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An unusual oral manifestation of type 1 neurofibromatosis: A case report and review of literature

Neurofibroma is a benign peripheral nerve sheath tumor and is the most frequent tumor of neural origin. Its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type 1 (NF1; a common hereditary disease occurring in one out of every 3000 births). The diagnosis can sometimes...

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Detalles Bibliográficos
Autores principales: Shekar, Vandana, Rangdhol, Vishwanath, Baliah, W. John, Thirunavukarasu, Sivasankari
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4367056/
https://www.ncbi.nlm.nih.gov/pubmed/25810680
http://dx.doi.org/10.4103/0976-9668.149225
Descripción
Sumario:Neurofibroma is a benign peripheral nerve sheath tumor and is the most frequent tumor of neural origin. Its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type 1 (NF1; a common hereditary disease occurring in one out of every 3000 births). The diagnosis can sometimes be made at birth, while in others the diagnosis is made later in life after the appearance of additional clinical criteria. Majority of the solitary neurofibromas are sporadic, while a few are associated with NF1 syndrome. Oral hard and soft tissue are affected by the tumor; however, the tongue is the most affected site. Gingival neurofibroma is an uncommon oral manifestation of NF. Here, we report a rare case of gingival neurofibroma in NF1 patient. One of the most feared complications of NF1 is its transformation into neurofibrosarcoma, which bears a very poor prognosis. Treatment of neurofibroma is surgical resection.