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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As a direct consequence, physical deterioration was also found by his parents.
B Preoperative panorex X-ray showed a characteristic radiographic feature of familial gigantiform cementoma FGC with well-circumscribed radiopaque areas involving all quadrants of the jaw, with mandible being more severely damaged. Clinical features demonstrating expansion of the left maxilla by a firm mass.
The lesions had an early age of onset and developed slowly. Citations Publications citing this paper. Case report A 6-year-old female presented with a main complaint of a painless maxillary swelling which her parents noticed 8 months earlier. In the affected family members, all 4 quadrants were involved. A CT image showed a spherical lobular radio-opaque mass measuring 4.
According to the latest World Health Organization WHO classification of cemento-osseous dysplasias CODsFGC is generally characterized by rapid osseous expansion involving all 4 jaw quadrants with predilection for young patients. Infected lesions and those presenting with progressive expansion are generally removed surgically through enucleation.
Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: D Postoperative view of patient after d v.
Familial gigantiform cementoma: classification and presentation of a large pedigree.
They found no definite evidence of familial occurrence. From Wikipedia, the free encyclopedia. There was a presumably affected male in an earlier generation who had an affected son. No history of similar lesions in the family of the patient was obtained.
Closer examination of his family pedigree verified our concern of a long-standing phenomenon of multiple fractures accompanying FGC in jaws. Ellis cementoja, Derek G.
Distinguishing features gigwntiform focal cemento-osseous dysplasia and cemento-ossifying fibromas. Compared with other 3 CODs, FGC takes on a really unique and aggressive form of behavior that is not supposed to be clinically approached in the existing framework of classification.
From This Paper Figures, tables, and topics from this paper. Loss of lamina dura, decreased skull bone density, and sporadic bony defects all served as convincing evidence of the serious osseous fragility.
In our patient the radiological, macroscopic and microscopic appearances are characteristics of a late-stage OD. According to the World Health Organization classification of osseous dysplasias ODFGC is generally regarded as an odontogenic lesion that shares a same periodontal ligament origin with focal, periapical.
Based on findings garnered from CT, it was noteworthy that 2 big circular deficits could be easily detected on both sides of the iliac bones.
A provisional diagnosis of an osteoma was made and the cementoka was enucleated through the buccal cortical plate. To sum up, our unusual radiographic and clinical findings of FGC give rise to a renewed understanding and a broad change to the stereotypic definition.
A radiolucent margin is present around most of the lesion. E Postoperative panorex X-ray showed vascularized iliac bone flap for reconstruction. Medunsa CampusSouth Africa.
He surmised that this phenomenon was merely because of genetic heterogeneity and not every case would develop such obvious concurrence of FGC and fractures. Osseous dysplasias ODs are generally slow growing, non-expansive, fibro-osseous jaw lesions of unknown aetiology. A The adolescent cemnetoma with a huge mass extending along the mandible body.
Familial Gigantiform Cementoma
Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity. December Learn how and when to remove this template message. The non-expansive Cementkma are not treated when asymptomatic and biopsy is ill-advised owing to the risk of infection.
A clinicopathologic study of 44 cases and review of the literature with special emphasis on recurrence. Radiographic examination revealed a well-circumscribed lobular radio-opaque mass surrounded by a radiolucent margin. Obliteration of the maxillary sinus and expansion of the maxillary bony cortex was noted. ArmstrongPeter Dirks Journal of neurosurgery.