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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Gabali Journal of oral and maxillofacial surgery….

Views Read Edit View history. 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.

Therefore, we agree with Noffke et al 14 and Finical 15 in resorting to a complete resection of FGC with curative purposes whenever feasible. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome. The process is often expansive, resulting in malposition of teeth with multiple impacted teeth.

Dentistry stubs Rare cancers. Familial gigantiform cementoma with Ehlers – Danlos syndrome: Nevertheless, in terms of his description, the progression of disease and related underlying causes has not been clarified. A The adolescent patient’s aunt who had received surgery nonvascularized iliac bone reconstruction 30 years ago now gigantoform of anterior maxillary mass with chronic infection.

Among all the reports available, Rossbach et al 3 was the first to postulate the correlation of a brittle bone disorder with FGC.


All these results came out with no marked aberrance. In such scenarios, extensive resection and free-flap reconstruction are required to achieve a better outcome. The lesions had an ggigantiform age of onset and developed slowly.

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D Postoperative view of patient after bilateral mandibulectomy. Cemento-osseous dysplasia of the jaw bones: Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: For sake of differential diagnosis with hyperparathyroidism-jaw tumor HPT-JT syndrome, 4 several laboratory analyses, which included serum parathyroid hormone PTHphosphate levels, calcium levels, and alkaline phosphatase ALP activity, were undertaken accordingly.

Gigantiform Cementoma, Familial Search for additional papers on this topic. Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity.

Gigantiform cementoma – Wikipedia

Please consider making a donation now and fmailial in the future. Abstract Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion.

Some of his consanguineous relatives bore the same burden of fractures during pubertal period. Gigantiform cementoma is a rare, autosomal dental tumor. Autosomal dominant gigantiform cementoma associated with bone fractures.

Unsourced material may be challenged and removed. Arteriovenous malformation of the scalp with cerebral steal. Journal List Medicine Baltimore v. By clicking accept or continuing to use the site, you agree to the terms outlined in our Privacy PolicyTerms of Serviceand Dataset License.


Closer examination of his family pedigree verified our concern of a long-standing phenomenon of multiple fractures accompanying FGC in jaws. His previous surgical history was remarkable and extensive. Pathology and Genetics of Head and Neck Tumours. The knowledge concerning FGC has been broadened for the recent proposition of the possible correlation with polyostotic diaphyseal cementomaa.


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Familial Gigantiform Cementoma

Surgery with recontouring and shaving intent was initially attempted in a local institution about 7 months ago, but in vain. 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. The disorder is usually not expansile, as is familial gigantiform cementoma.

In the family reported by Young et al. C Lateral view of FGC. Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cemento-osseous-fibrous lesion. Clinicopathologic presentation of 3 cases. Gigantiform cementoma in a child. Multiquadrant expansile fibro-osseous lesion in a juvenile.

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

Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis. Besides, it follows an autosomal dominant inheritance pattern with divergent phenotypic expression. CC ]. From Wikipedia, the free encyclopedia. Gigantiform cementoma is characterized by diffuse radioopaque masses scattered throughout the jaws.