Odontogenic myxoma in an adolescent with tuberous sclerosis

Tuberous sclerosis is a hamartoneoplastic syndrome characterised by early onset of convulsive seizures with mental retardation. Oral abnormalities, including enamel hypoplasia and mucosal fibromas, have been previously reported. We report here a 17-year-old girl with this disorder who had received repeated dental trauma. She presented with a swelling associated with a traumatised maxillary central incisor that suggested a diagnosis other than the odontogenic myxoma found at operation. Odontogenic myxoma has not previously been reported in this condition; it may represent an unusual phenotypic expression of the genetic defect understood to determine tuberous sclerosis, or it may be an unrelated coincidental finding.