Abstract

Tuberous sclerosis is a rare disease with dominant autosomal inheritance. The most important clinical findings are epileptic seizures, mental retardation, and hamartomatous tumors; it may affect the skin, nervous system, heart, kidneys, and oral mucosa. Among the intraoral findings, enamel pits, cleft lip and palate, fibrous papular lesions, and bone cysts may be present. We present the case of a 20-year-old man with dermatological and neurological signs, with a history of increased volume in the anterior region of the maxilla, with a 13-year course. The surgical procedure involved an ambulatory approach under local anesthesia. The lesion had a fibrous aspect, white-colored, with cleavage points, rendering complete enucleation possible. A histopathological analysis revealed weakly vascularized fibrous tissue with calcifications, with a subjective result of desmoplastic fibroma.

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