Cherubism

Cherubism, a clinicopathological entity of genetic origin, predominantly affects the mandible, with maxillary involvement to a lesser degree. It is usually recognized roentgenographically and clinically between the ages of 18 months and 2 years; the condition then develops rapidly for two to three years, followed by a period of stasis and then regression. Symmetrical, multilocular cysts are seen radiologically. Histopathologically, vascular fibrous tissue containing multinucleated giant cells is seen. With development of adult characteristics, the face develops a more normal appearance. Between 20 and 30 years of age, radiolucent areas are filled with bone of a dense granular pattern, and the jaw assumes a more normal contour. Active treatment in mild cases is not necessary. Surgery is indicated only when appearance or function can be improved by removal of bone with preservation of dentition where possible.