Abstract: PURPOSE : To summarize the clinical features of patients with familial gigantiform cementoma (FGC). METHODS : We performed a retrospective study in a series of 11 patients. The hematology indicators, as well as the clinical and radiographic findings in different stages were analyzed. RESULTS : During initial onset, the lesions involved in the patients' jaws. CT scans showed multiple radiolucent swellings in the mandible and maxilla. The level of blood alkaline phosphatase was raised slightly. The bone mineral content was almost within normal limits. During rapid expansion, a marked enlargement of the masses with severe facial deformity and malocclusion was present. Patients always sustained multiple pathological long bone fractures. CT demonstrated multiple, expansile, mixed radiolucent-radiopaque masses in all four quadrants of their jaws. Patients had raised blood alkaline phosphatase and the bone mineral content decreased in this stage. During growth suppression phase, the lesions showed no apparent sign of increased expansion. CT scans revealed large radiolucent swellings. The blood alkaline phosphatase returned to normal range and the bone mineral content increased. CONCLUSIONS : In different phases, patients with FGC have special clinical characteristics. These features are extremely helpful in assessing the growth potential of FGC, as well as in formulating the treatment strategies.

Key words: Familial gigantiform cementoma, Clinical phase, Clinical feature