Abstract

ACOSTA FERIA, M. et al. Desmoplastic fibroma of the jaw associated with tuberous sclerosis: Literature review and case report. Rev Esp Cirug Oral y Maxilofac [online]. 2008, vol.30, n.2, pp.107-114. ISSN 2173-9161.

Tuberous sclerosis is a congenital anomaly of embryonic development with an autosomal dominant inheritance. It is characterized by the presence of neurologic and dermatologic disorders and mental retardation. It can affect other organs and systems and produce orofacial manifestations. Dental enamel defects are the most frequent intraoral lesion. Bone lesions rarely occur in the upper jaw. Desmoplastic fibroma is an infrequent, slow-growing, locally aggressive intraosseous fibrous tumor that rarely is associated with tuberous sclerosis. We report the clinical case of a 33-year-old man with tuberous sclerosis and a jaw lesion diagnosed as desmoplastic fibroma. The diagnostic methods, clinical presentation, and treatment are discussed.

Keywords : Tuberous sclerosis; Desmoplastic fibroma; Bourneville- Pringle disease; Oral manifestations; Jaw.

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