Familial gigantiform cementoma with brittle bone disease. It is for these reasons that a minimum of two eadiology shown in figure most injuries to the middle third of the face are from the front, forcing part or parts of the facial in addition, the knowledge required by the skeleton downwards and backwards along the clinician can again be summarized as follows. It is found most commonly in the mandible in the region of the lower molar teeth, occurring between the ages of 8 to 30 in both sexes with equal frequency. Familial gigantiform cementoma with ehlers danlos syndrome. This is an exceedingly rare tumor with only a handful of documented cases worldwide.
Abstract the gigantiform cementoma gc is an extremely rare, benign condition of fibro. There was no indication in the patients case file whether these were familial or nonfamilial. The demographic and clinical information was obtained from each individual patients clinical files. Gigantiform cementoma is a rare, benign fibrocementoosseous disease of the jaws, seen most frequently in young girls. Familial gigantiform cementoma is an exceedingly rare but distinct subtype of cementoosseousfibrous lesion. Cureus nonfamilial cherubism with bilateral maxilla and. These include a case of florid cementoosseous dysplasia previously called gigantiform cementoma. It is occasionally manifested before the age of two years. What links here related changes upload file special pages permanent link page. Wikiproject dentistry rated stubclass, lowimportance this article is supported by wikiproject dentistry.
Epulis literally, on the gingiva is a general term for any gingival or alveolar tumor i. Radiographically, it typically presents as multiquadrant, expansile, mixed radiolucentopaque lesions that cross the midlines of the jaws. Bone dysplasias are characterized by the replacement of normal bone with fibrous tissue containing abnormal bone or cementum. Familial gigantiform cementoma is a rare benign fibrocementoosseous lesion of the jaws that can cause severe facial deformity. It is characterized by notable clinical bilateral swelling of the cheeks due to a bony enlargement of the jaws that impart a characteristic cherubic look. Undocumented radiographic changes and related bone metabolism disorder are herein hypothesized and discussed. Cancrum oris pdf noma is an opportunistic infection promoted by extreme poverty. Cherubism is a selflimiting nonneoplastic autosomal dominant fibroosseous syndrome of the jaws. Radiologically, it closely resembles fibrous dysplasia and other giant cell lesions of the mandible.
According to the classification system introduced by the revised world health organization who guidelines, cementoosseous dysplasia is categorized as a form of neoplasm or other bonerelated lesion. All the lesion were situated within the alveolar process of the front mandibular teeth lower incisor teeth. Florid osseous dysplasia in orientals sciencedirect. It is benign, but without intervention it can result in severe disfigurement of the jaw. Original article florid osseous dysplasia coexisting with. Fibroosseous lesions refer to a collection of nonneoplastic intraosseous lesions that replace normal bone and consist of a cellular fibrous connective tissue within which nonfunctional osseous structures form. This is the first case report that describes nonfamilial gigantiform cementoma after the world health organisations classification of 2005. Abstractfamilial gigantiform cementoma is an exceedingly rare but distinct subtype of cementoosseousfibrous lesion. Cementicle cementoblastoma gigantiform cementoma eruption cyst epulis pyogenic granuloma congenital epulis gingival enlargement gingival cyst of the adult gingival cyst of the newborn gingivitis desquamative granulomatous plasma cell hereditary gingival fibromatosis. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with ehlersdanlos syndrome. West indian medical journal university of the west indies. Young sk1, markowitz nr, sullivan s, seale tw, hirschi r. 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.
The story of his life garnered much media attention when he was taken to taiwan for treatment. Eversole fibro osseous lesions are a diverse group of processes that are characterized by. Familial gigantiform cementoma is a rare autosomal dominant tumor that is benign but can result in. Five were documented over a 15year period 1980 1995. The variant known as gigantiform cementoma refers to a number of symptomatic lesions. Cementicle epjlis gigantiform cementoma eruption cyst epulis pyogenic granuloma congenital epulis gingival enlargement gingival cyst of the adult gingival cyst of the newborn gingivitis desquamative granulomatous plasma cell hereditary gingival fibromatosis hypercementosis hypocementosis linear gingival erythema necrotizing periodontal diseases. Benign fibroosseous lesions of the craniofacial complex a. Familial gigantiform cementoma fgc is an uncommon benign, fibrocementoosseous lesion of the jaws, which appears to be transmitted as an autosomal dominant trait with variable phenotypic. Familial gigantiform cementoma is a rare autosomal dominant tumor that is benign but can result in disfigurement of the facial skeleton. Benign fibroosseous lesions of the craniofacial complex are represented by a variety of disease processes that are characterized by pathologic ossifications and calcifications in association with a hypercellular fibroblastic marrow element.
This condition has also been classified as gigantiform cementoma, chronic sclerosing osteomyelitis, sclerosing osteitis, multiple estenosis and sclerotic cemental. Florid osseous dysplasia in orientals loh funchee, bds, mds, msc, am,and yeo jinn fei, bds, msc, mds, am,singapore, republic of singapore national university of singapore florid osseous dysplasia has been described as a condition that characteristically affects the jaws of middleaged black women. Gigantiform cementoma is a very rare lesion in the jaw. Original article florid osseous dysplasia coexisting with focal osseous dysplasia in the jaw. If you want to participate andor join, please visit the project page, or ask questions on the project talk page. The mouth constitutes a sort of no mans land between the domain of the dermatologist and that of the dentist, and the boundaries of their respective fields a. Novemthree siahaan november 3, 1998 september 14, 2005 was a young indonesian boy who had the rare condition gigantiform cementoma. Gigantiform cementoma of the jaws gigantiform cementoma of the jaws thompson, stevan h altini, mario 19891101 00. Both sporadic cases of gc and those with an autosomal dominant pattern of inheritance have been reported in the english literature. Pdf familial gigantiform cementoma is an exceedingly rare but distinct subtype of cementoosseousfibrous lesion. Pdf familial gigantiform cementoma is an exceedingly rare but distinct subtype of. The etiology is also unclear, but it is believed to have.
Three of the initial cases were histologically diagnosed as gigantiform cementoma. Very few cases of gigantiform cementoma have been reported, and those associated with a positive family history are especially rare. Gigantiform cementoma in a child dentomaxillofacial. Eagle syndrome hemifacial hypertrophy facial hemiatrophy oral manifestations of systemic disease. The difficulty of making a definitive diagnosis on histological grounds is discussed. Pdf familial gigantiform cementoma is an exceedingly rare but distinct. The gigantiform cementoma gc is an extremely rare, benign condition of fibrocementoosseous origin that is noted in the jaws, more commonly in younger individuals. Non familial cherubism jain v, gamanagatti s r, gadodia a, kataria p, bhatti s s abstract cherubism is a disease that usually affects the jaws in the paediatric population, mostly below five years of age.
This article describes the clinicopathological features of 28 lesions classified as gigantiform cementoma. Familial gigantiform cementoma fgc is a distinct and uncommon fibrocementoosseous lesion with unknown etiology. Gigantiform cementoma is a rare, autosomal dental tumor. 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. The etiology is also unclear, but it is believed to have a genetic component. Nasopalatine duct median mandibular median palatal traumatic bone osteoma osteomyelitis osteonecrosis bisphosphonateassociated neuralgiainducing cavitational osteonecrosis osteoradionecrosis osteoporotic bone marrow defect pagets disease of bone periapical abscess. It has an autosomal dominant mode of inheritance, but varies in its. However, there were only few examples in the literatures in which the familial pattern had been confirmed. The etiology is also unclear, but it is believed to. Fibro osseous lesions classifications, pathophysiology. Clinical, radiographic, biochemical and histological findings of florid.
We present an adolescent case with recurrent familial gigantiform cementoma who received surgical. An epulis granulomatosa is a granuloma which grows from an extraction socket the hole left after a tooth has been. Undocumented radiographic changes and related bone metabolism disorder are herein. It is usually observed as a benign spherical mass of hard tissue fused to the root of a tooth.
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