Oral Surg Oral Med Oral Pathol. Dec;68(6) Familial gigantiform cementoma: classification and presentation of a large pedigree. Young SK(1). Gigantiform cementoma is a rare, benign fibro-cemento-osseous disease of the jaws, seen most frequently in young girls. Radiographically, it typically presents. PDF | Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Undocumented.

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No history of similar lesions in the family of the patient was obtained. The skin over the lesion was freely movable and no nerve fall-out was noted. Clin Radiol ; Focal OD is a solitary lesion affecting a single area in the jaw crmentoma and mainly the posterior quadrant of the jaws.

It is benign, but without intervention it can result in severe disfigurement of the jaw. Circumstantial evidence and thorough reasoning were presented in his article for differential diagnosis with other gigantifogm mistaken diseases, such as Paget disease, cemento-ossifying fibromas and osteogenesis imperfecta.

National Center for Biotechnology InformationU. Cementoma Search for additional papers on this topic. FGC is uncommon, occurs predominantly in young patients and has the potential to cause considerable jaw expansion. Clinical features demonstrating expansion of the left maxilla by a firm mass.

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All these results came out with no marked aberrance. Abstract Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion.

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In our patient the radiological, macroscopic and microscopic appearances are characteristics of a late-stage OD. Pediatr Blood Cancer ; Before referral, he had experienced 4 times of fractures in bilateral lower extremities within 4 years. Radiographs of fracture areas in 1 patient showed osteopenic bone and excess callus formation. Fibro-osseous lesions of the head gigantifomr neck.

In concurrence with the boy and his parent’s wishes, we decided to perform a bilateral segmental mandibulectomy and to tentatively reconstruct the corresponding defect with vascularized iliac bone cementoja. Looking For More References? Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. Clinical Synopsis Toggle Dropdown. They found no definite evidence of familial occurrence. C Lateral view of FGC. Topics Discussed in This Paper.

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Familial gigantiform cementoma: classification and presentation of a large pedigree.

In the affected family members, all 4 quadrants were involved. Expert curators review the literature and organize it to facilitate your work. Suspicious of similar osseous problems in other anatomic gigantifor, we then recommended a pelvic computer tomography CT scan to the patient.

Support Center Support Center. Autosomal dominant gigantiform cementoma associated with bone fractures. Diagnostic Histopathology ; From Wikipedia, the free encyclopedia. B Preoperative panorex X-ray showed a characteristic radiographic feature of familial gigantiform cementoma FGC with well-circumscribed gigqntiform areas involving all quadrants of the jaw, with mandible being more severely damaged.

A provisional cementima of an osteoma was made and the lesion was enucleated through the buccal cortical plate. Familial florid cemento-osseous dysplasia — a case report and review of the literature. Some of his consanguineous relatives bore the same burden of fractures during pubertal period.