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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E Postoperative panorex X-ray showed vascularized iliac bone flap for reconstruction. Familial gigantiform cementoma FGC is a distinct and uncommon fibro-cemento-osseous lesion with unknown etiology. Medunsa Campus gigzntiform, South Africa. A provisional diagnosis of an osteoma was made and the lesion was enucleated through the buccal cortical plate. Focal OD is gigantifkrm solitary lesion affecting a single area in the jaw bone and mainly the posterior quadrant of the jaws.

Clinicopathologic presentation of 3 cases. Macdonald Oral surgery, oral medicine, oral pathology and….

Familial Gigantiform Cementoma

Head Neck ; Gigantiform cementoma is a rare, autosomal dental tumor. In our patient the radiological, macroscopic and microscopic appearances are characteristics of gigantifrm late-stage OD. Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis.

Familial occurrence of periapical cemental dysplasia. Some of his consanguineous relatives bore the same burden of fractures during pubertal period. Infected lesions and those presenting with progressive expansion are generally removed surgically through enucleation.


E Technetium scintigraphy showed increased tracer uptaker in the chin area. The knowledge concerning FGC has been broadened for cementlma recent proposition of the possible correlation with polyostotic diaphyseal fractures.

J Neurosurg Pediatr ; 7: Before referral, he had experienced 4 times of fractures in bilateral lower extremities within 4 years. Fibro-osseous lesions of the face and jaws.

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

Ggiantiform, we agree with Noffke et al 14 and Finical 15 in resorting to a complete resection of FGC with curative purposes whenever feasible. Gabali Journal of oral and maxillofacial surgery…. The most famous case is of Novemthree Siahaan who died on September 15,a young Indonesian boy from Batam Island who received medical care in Haulien, Taiwan gigabtiform a Buddhist missionary from the Tzu Chi Foundationwhich was documented on the Discovery Health Channel.

Radiographic examination revealed a well-circumscribed lobular radio-opaque mass surrounded by a radiolucent margin. However, owing to such diversion of calcium supply, bone fragility was manifested in the other anatomic regions, especially in lower extremities. Osteomas are more common in the craniofacial bones than in other parts of the skeleton. By clicking accept or continuing to use the site, you agree to the terms outlined in our Privacy PolicyTerms of Serviceand Dataset License.

Autosomal dominant gigantiform cementoma associated with bone fractures.

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

Micrograph demonstrating the bony mass showing a lobular appearance with small marrow spaces arrows. A diagnosis of a gigantiform cementoma was made. The process is often expansive, resulting in malposition of teeth with multiple impacted teeth.

Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: Besides, it follows an autosomal dominant inheritance pattern with divergent phenotypic expression.

Distinguishing features of focal cemento-osseous dysplasia and cemento-ossifying fibromas. Based on findings garnered from CT, it was noteworthy that 2 big circular deficits could be cemeentoma detected on gigantifrom sides of the iliac bones.

His previous surgical history was remarkable and extensive. 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. Unfortunately, it is not free to produce.

Familial Gigantiform Cementoma

A bonus to all MIMmatch users is the option to sign up for updates on new gene-phenotype relationships. Suspicious of similar osseous problems in other anatomic locations, we then recommended a pelvic computer tomography CT scan to the patient. Topics Discussed in This Paper.