FAMILIAL GIGANTIFORM CEMENTOMA PDF

March 23, 2020 By admin

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.

Author: Zukazahn Vicage
Country: Brazil
Language: English (Spanish)
Genre: Personal Growth
Published (Last): 8 May 2005
Pages: 329
PDF File Size: 11.96 Mb
ePub File Size: 4.65 Mb
ISBN: 851-1-21997-707-8
Downloads: 73696
Price: Free* [*Free Regsitration Required]
Uploader: Kajit

This dentistry article is a stub. Dentistry stubs Rare cancers.

Autosomal dominant gigantiform cementoma associated with bone fractures. Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. There was a presumably affected male in an earlier generation who had an affected son. InMoshref et al 8 again reported a FGC case series with frequent fracture history.

Histologically, the lesions were composed of benign, lobulated, calcified masses resembling cementum.

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

Noura AlsufyaniErnest W. The ethical approval was granted by the Institutional Clinical Research Supervision Committee of our hospital.

We present an adolescent case with recurrent familial gigantiform cementoma who received surgical intervention in our hospital. NarayanWilfried Wagner Oral surgery, oral medicine, oral pathology and…. The disorder is usually not expansile, as is familial gigantiform cementoma.

Cemento-osseous dysplasia of the jaw bones: Therefore, we present a case with gigantlform large FGC family history so afmilial to sketch a more detailed portrait of such ailment. Some of his consanguineous relatives bore the same burden of fractures during pubertal period. The feasibility of using fibular flaps was ruled out in view of his unfortunate history of frequent lower-extremity fractures.

Familial Gigantiform Cementoma

We are determined to keep this website freely accessible. All these fractures, which occurred in diaphyseal locations, were categorized to a minor-trauma or spontaneous causes owing to poor evidence of outside forces.

  DD 1351-2 PDF

Florid osseous dysplasia, which is histologically similar, may be distinct. Support Center Support Center. In the family reported by Young et al. For the latest visit to our clinic 2 months ago, the outcome in the neomandible region was desirable, and in parallel, the recent DEA test result took a favorable turn as BMD had increased to To keep both practitioners and patients informed of the overall bony changes and corresponding risks of fractures, we contended that DEA should be routinely tested since initial clinic visit.

Pediatr Blood Cancer ; Apart from typical multiquadrant and expansile abnormalies involving both jaws, he also suffered from several times of fractures in lower extremity. Among all the reports available, Rossbach et al 3 was the first to postulate the correlation of a brittle bone disorder with FGC. Gigantiform cementoma is characterized by diffuse radioopaque masses scattered throughout the jaws.

Radiographs of fracture areas in 1 patient showed osteopenic bone and excess callus formation. Diagnostic Histopathology ; Topics Discussed in This Paper. Showing of 15 references. Panoramic radiography showing periapical lesions and missing teeth. A The adolescent patient with a huge mass extending along the mandible body.

To further identify the cemenyoma 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. Gigantiform Cementoma, Familial Search for additional papers on this topic. World Health Organization Classification of Tumours: From This Paper Figures, tables, and topics from this paper.

Views Read Edit View history. Despite the paucity of information regarding FGC, DEA, as we believe, carries diagnostic and therapeutic implications, along with other radiographic examinations. This is fmailial open access article distributed under the Creative Commons Attribution License 4.

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

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

  JURNALUL UNEI TINERE FETE ANNE FRANK PDF

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.

Report of a case documented with computed tomography and 3D familil. Plast Reconstr Surg ; A bonus to all MIMmatch users is the option to sign up for updates on new gene-phenotype relationships. Cementlma 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.

It is benign, but without intervention it can result in severe disfigurement of the jaw. Gigwntiform article has been cited by other articles in PMC. From Wikipedia, the free encyclopedia.

Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. As far as our knowledge is concerned, this pathognomonic trait thus necessitates timely and appropriate surgical treatment to prevent dementoma terrible clinical scenarios from spinning out of control. Incomplete famolial or shave-off contouring is not advised because it may cause a possible aggravation or reactivation of rapid FGC growth.

Prosthodontic treatment for edentulous patients, vol 1. Looking For More References?

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 flaps. B Preoperative panorex X-ray showed a characteristic radiographic feature of fammilial gigantiform cementoma FGC with well-circumscribed radiopaque areas involving all quadrants of the jaw, with mandible being more severely damaged.