PDF | Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Undocumented. Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. Confusion exists about the. Familial gigantiform cementoma is a rare benign fibrocemento-osseous lesion of the jaws that can cause severe facial deformity. It has an.

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Circumstantial evidence and thorough reasoning were presented in his article for differential diagnosis with other easily mistaken diseases, such as Paget disease, cemento-ossifying fibromas and osteogenesis imperfecta. Periprosthetic bone mineral density changes after unicondylar knee arthroplasty. D Postoperative view of patient after bilateral mandibulectomy. During the rapid growth phase of FGC in mandible and maxilla, the other bones, especially long bones in lower extremities, were simultaneously undergoing a calcium absorption or transportation problem, as was reflected in the osteoporotic radiographic changes in our adolescent patient.

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Author information Article notes Copyright and License information Disclaimer. She is currently under treatment, which she may need to continue until her growth stops in her early 20s.

Among all the reports available, Rossbach et al 3 was the first to postulate the correlation of a brittle bone disorder with FGC. Citations Publications citing this paper. Nevertheless, in terms of his description, the progression of disease and related underlying causes has not been clarified. Topics Discussed in This Paper. References Publications referenced by this paper.


Besides, his pelvis was also considered to be susceptible to fractures because both cortical and trabecular bones were undergoing a progeric or osteopenic conversion signifying an unexpected calcium loss. Citations Publications citing this paper.


Florid osseous dysplasia, which is histologically similar, may be distinct. Gigantiform Cementoma, Familial Search for additional papers on this topic. Head Neck ; Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: The radiographic evaluations of lower limb revealed decreased bone density and thinner- or void-cortex structures around fracture regions. They observed the disorder in a mother, 2 of her daughters, and a son.

Familial gigantiform cementoma with Ehlers – Danlos syndrome: National Center for Biotechnology InformationU. World Health Organization Classification of Tumours: The process is often expansive, resulting in malposition of teeth with multiple impacted teeth. The knowledge concerning FGC has been broadened for the recent proposition of the possible correlation with polyostotic diaphyseal fractures.

Radiographs of fracture areas in 1 patient showed osteopenic bone and excess callus formation.

She was yet no exception given her own narratives of femur fracture during adolescence. You can help Wikipedia by expanding it.

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

Cementom, not every FGC case will present such evident array of disorganized conditions. A The adolescent patient with a huge mass extending along the mandible body.

Fibro-osseous lesions of the face and jaws. Showing of 20 references. Gigantiform cementoma is a rare, autosomal dental tumor. The lesions had an early age of onset and developed slowly. From This Paper Figures, tables, and topics from this paper.

By using this site, you agree to the Terms of Use and Privacy Policy. Closer examination of his family pedigree verified our concern of a long-standing phenomenon of multiple fractures accompanying FGC in jaws.


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 through a Buddhist missionary from the Tzu Chi Foundationwhich was documented on the Discovery Health Channel. From Wikipedia, the free encyclopedia. Articles lacking sources from December All articles lacking sources Infobox medical condition All stub articles.

Therefore, we present a case with a large FGC family history so as to sketch a more detailed portrait of such ailment. From This Paper Figures, tables, and topics from this paper.

Apart from that, it is still debatable about the timing of surgical intervention for FGC patients. To further identify the specific reasons for multiple fractures alongside these osseous changes previously described, whole-body dual-energy absorptiometry DEA measuring bone mineral density BMD was then offered under permission of the patient and his parents.

Topics Discussed in This Paper. Surgical removal of the affected bone is needed, and has to be followed by reconstruction. Autosomal dominant gigantiform cementoma associated with bone fractures. Macdonald Oral surgery, oral medicine, oral pathology and…. Fibro-osseous lesions of the head and neck. Report of a case documented with computed tomography and 3D imaging.

Surgery with recontouring and shaving intent was initially attempted in a local institution about 7 months ago, but in vain.