Gigantiform cementoma

Gigantiform cementoma is a rare, benign fibro-cementoosseous disease of the jaws. It is characterized by formation of massive sclerotic masses of disorganized mineralized material. Both sporadic and familial occurrence has been reported. The etiopathogenetic mechanism of gigantiform cementoma is unknown.

The term gigantiform cementoma has been used synonymously with the terms sclerotic cemental masses, florid osseous dysplasia, multiple enostoses and diffuse sclerosing osteomyelitis.

Clinical features

• Gigantiform cementoma is an autosomal dominant disorder having high penetrance and variable expressivity. No sex predilection has been observed.
• It typically presents as a slow-growing, multifocal/multiquadrant and expansile lesions involving both the jaws.
• The lesions are usually seen in younger age, although older patients have also been reported.
• The characteristic clinical manifestation is of a painless maxillary and mandibular swelling with associated facial deformity.
• Tooth impaction, malpositioning of teeth and malocclusion are also seen associated with the swelling.
• The enlargement of the swelling finally stops during the 5th decade.

Radiographic features

• Radiographically, gigantiform cementoma exhibits multiple circumscribed, expansile and lobular mixed radiolucent radiopaque lesions that usually cross the midlines of the jaws.
• Sometimes large masses are seen in posterior areas of the jaws and the lesions combining in the midline. This suggests that lesions might have started as separate posterior masses; however, with anterior progression they became confluent and crossed the midlines of the jaws.

Histopathologic features

• It is characterized by variable degrees of cellularity and variation in the amount and size of mineralized deposits.
• The mineralized component is present as admixture of small and large psammomatoid and spherical hematoxylinophilic, cementum-like calcified deposits and irregular bony trabeculae in a fibroblastic proliferative background.
• The fibrous stroma may show fascicular or storiform patterns with spindle-shaped or stellate-shaped fibroblasts.
• The lesions are generally hypovascular, with occasionally focally rich vascular areas exhibiting aggregates of thick walled small blood vessels.
• Mitoses, hyperchromatism and pleomorphism are not seen.

Management and prognosis

The surgical management of gigantiform cementoma is usually difficult because of the extensive involvement of the jaws with these tumors. Surgical inaccessibility, particularly in the posterior regions of the jaws seems to be responsible for recurrence of the tumors. These lesions seem to have a tendency toward recurrence when they are treated with incomplete surgical removal. It is therefore recommended that these lesions should be managed with conservative but complete surgical excision whenever feasible.

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Source: Data courtesy of the U.S. National Library of Medicine. Since the data might have changed, please query MeSH on Gigantiform cementoma for any updates.

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