Korean J Oral Maxillofac Radiol.  2006 Sep;36(3):157-162.

Familial gigantiform cementoma

• Han WJ
• Kim EK

Abstract

Familial gigantiform cementoma is a rare fibro-cemento-osseous disease of the jaws which appears to be transmitted as an autosomal dominant trait with variable expressivity of the phenotype. A 7-year-old girl visited DKUDH complaining of the painless facial deformity. Clinically, significant facio-lingual expansion was observed at the left maxilla, left mandibular body and symphysis portion. Malposition of lower anterior teeth was found. Panoramic radiograph and CT scan showed the extensive expansile mixed lesion at maxilla and mandible. Bone scan revealed hot spot at the maxilla and left side of mandible. Histologic examination revealed moderately dense fibrous connective tissue with scattered masses resembling cementum. The patient's mother had a history of the mandibular resection due to benign tumor. Her younger brother had buccal expansion of right mandible. We report our finding of a family that has exhibited clinical, radiographic and histologic findings consistent with the familial gigantiform cementoma.

Keyword

Odontogenic Tumors; Cementoma

MeSH Terms

Cementoma*
Child
Congenital Abnormalities
Connective Tissue
Dental Cementum
Female
Humans
Jaw
Mandible
Maxilla
Mothers
Odontogenic Tumors
Phenotype
Siblings
Tomography, X-Ray Computed
Tooth