Focal od is a solitary lesion affecting a single area in the jaw bone and mainly the posterior quadrant of the jaws. Gigantiform cementoma affecting a caucasian family sciencedirect. Lesions are composed of benign, lobulated, calcified masses resembling cementum. It has an autosomal dominant mode of inheritance, but varies in its. However, periapical cemental dysplasia is definitely a reactive process, whereas cementoma is thought to be a true neoplasm, and cementifying fibroma and gigantiform cementoma are probably also tumorlike lesions. A cementoma is not an uncommon lesion to present in the jaws. It has been unclear whether gigantiform cementoma should be accorded the status of a separate entity. Familial gigantiform cementoma with ehlers danlos syndrome. Gigantiform cementoma in a child dentomaxillofacial.
Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with ehlersdanlos syndrome. Gigantiform cementoma is a very rare lesion in the jaw. The difficulty of making a definitive diagnosis on histological grounds is discussed. Identification of a col1a2 mutation in one patient was a. It will be seen from the chart that these are distinct lesions in respect of location, sex prediliction, age. Case report of an unusual clinical manifestation and possible mechanism. Radiologic and pathologic characteristics of benign and. The university of oklahoma colleges of dentistry and medicine very few cases of gigantiform cementoma have been reported, and those associated with a. A rare, benign, slow growing, multifocal, and expansile lesion of the jaws. Gigantiform cementoma is a rare, benign fibrocementoosseous disease of the jaws, seen most frequently.
True fgc with familial history is extremely rare and there has been no literature regarding the radiological followup of fgc. Familial gigantiform cementoma fgc is a rare autosomal dominant, benign fibrocementoosseous lesion of the jaws that can cause severe facial deformity. Other effects that set its power or toughness to specific values that start to apply after gigantiform becomes attached to it will overwrite gigantiform. Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma. The full text of this article hosted at is unavailable due to technical difficulties. The objective of this report is to present an unusual case of a gigantiform cementoma manifesting with gross expansion of the maxilla in a 6yearold black female. Autosomal dominant gigantiform cementoma associated with bone fractures. Pdf familial gigantiform cementoma is an exceedingly rare but distinct subtype of cementoosseousfibrous lesion. Bone dysplasias are characterized by the replacement of normal bone with fibrous tissue containing abnormal bone or cementum. Familial gigantiform cementoma fgc is a rare benign autosomal dominant fibrocementoosseous lesion generally limited to the facial bones, typically in the. Both familiar and sporadic cases have been reported. The etiology is also unclear, but it is believed to have. Gigantiform cementoma is a rare, autosomal dental tumor.
Although the gigantiform cementoma is considered by some authors to be a true neo plasm, radical removal, if possible at all, is not considered to be necessary. Cementicle cementoblastoma gigantiform cementoma eruption cyst epulis pyogenic granuloma congenital epulis gingival enlargement gingival cyst of the adult. The gc is a painless, well circumscribed tumor with limited growth, which generally requires surgical treatment. Gigantiform cementomaan unusual incidental finding the. The gigantiform cementoma gc is an extremely rare, benign condition of fibrocementoosseous origin that is noted in the jaws, more commonly in younger individuals. The mother and her children presented with deformities of the jaws, abnormalities of the long bones, and pre. This may emerge as the result of progressive alveolar atrophy under a denture or after extraction of teeth in the affected area involved 5. Differentiating early stage florid osseous dysplasia from periapical.
Confusion exists about the relationship of gigantiform cementoma to florid osseous dysplasia, cementifying fibroma, and diffuse chronic sclerosing osteomyelitis. Gigantiform cementoma is a rare, benign fibrocementoosseous disease of the jaws, seen most frequently in young girls. This is an exceedingly rare tumor with only a handful of documented cases worldwide. Case report gigantiform cementoma in a child cee noffke,1, sp ngwenya2, n nzima1, ej raubenheimer2 and nb rakgwale1 1radiology and 2oral pathology, school of oral health sciences, faculty of health sciences, university of limpopo, limpopo province, south africa the objective of this report is to present an unusual case of a gigantiform cementoma. Familial gigantiform cementoma with brittle bone disease. Both sporadic cases of gc and those with an autosomal dominant pattern of inheritance have been reported in the english literature. Autosomal dominant gigantiform cementoma associated with. Pdf familial gigantiform cementoma is an exceedingly rare but distinct. Pdf familial gigantiform cementoma with ehlers danlos. Classification and presentation of a large pedigree stephen k. Unfortunately, in the present case there was no reliable information about a familial occurrence of gigantiform cementoma. In the affected family members, all 4 quadrants were involved. The gigantiform cementoma is classified by the world health organization who within the osseous dysplasias and its prevalence is very low.
Seale, phd,d and robert hirschi, dds, ms,e oklahoma city, okla. American family with a predominantly diaphyseal bone disease associated with familial gigantiform cementoma fgc, a disorder typically seen in caucasians. Gigantiform overwrites all previous effects that set the enchanted creatures power and toughness to specific values. Familial gigantiform cementoma is a very rare fibroosseous lesion of the maxillofacial bones characterized by early onset of multifocal. The gigantiform cementoma is an uncommon lesion that is usually observed as an incidental finding on the dental panoramic radiograph. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucentopaque lesions that cross the midlines of the jaws. For many years all the lesions designated by the terms above were classified simply as cementomas thoma 1970. Figure 6 from familial gigantiform cementoma with ehlers. Familial gigantiform cementoma fgc is a distinct and uncommon fibrocementoosseous lesion with unknown etiology. It is intended to analyse all the cases of benign fibro. Familial gigantiform cementoma is a rare benign fibrocementoosseous lesion of the jaws that can cause severe facial deformity. Florid cementoosseous dysplasia and osteomyelitis scielo.
It is benign, but without intervention it can result in severe disfigurement of the jaw. Ehlersdanlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementumlike masses throughout the jaws. Gigantiform cementoma is characterized by diffuse radioopaque masses scattered throughout the jaws. We describe four individuals of an africanamerican family with a predominantly diaphyseal bone disease associated with familial gigantiform cementoma fgc, a disorder typically seen in caucasians. Both affected children had swellings of the alveolar crest. Case report of an unusual clinical manifestation and possible mechanism related to calcium steal. Periapical cemental dysplasia fibro cementoma, sclerosing cementoma, periapical fibrous dysplasia pcd is a localized change in the normal bone metabolism that results in the replacement of the components of normal cancellous bone with fibrous tissue and cementum like material, abnormal bone or a mixture of two.
The term gigantiform cementoma has been used interchangeably with designations of other fibroosseous entities, yet its application should be restricted by. Here, we report a family with gigantiform cementomas, bone fractures, and autosomal dominant inheritance. Pdf familial gigantiform cementoma with brittle bone. The etiology is also unclear, but it is believed to. The gigantiform cementoma gc is an extremely rare, benign condition of fibro. According to the latest world health organization who classification of cementoosseous dysplasias cods, fgc is generally characterized by rapid osseous expansion involving all 4 jaw quadrants with predilection for young patients. Pdf familial gigantiform cementoma is an exceedingly rare but distinct subtype of. Osseous dysplasias ods are generally slow growing, nonexpansive, fibroosseous jaw lesions of unknown aetiology. In this article, we report our findings on a family that, over five generations, has exhibited clinical, radiographic, andor histologic findings consistent with the designation familial gigantiform cementoma. Followup ct findings of recurrent familial gigantiform. This paper intends to present clinical, radiologic, and histopathologic features of a case of gigantiform cementoma manifesting in a 14yearold boy, without any familial involvement. Molecular findings in maxillofacial bone tumours and its diagnostic. The lesion has been classified by pindborg et al 1971 as a benign odontogenic lesion most commonly seen.
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