Cherubism

Abstract

The nine known cases of cherubism in Denmark have been followed over a 7-year-period and were examined with regard to the clinical and radiographic appearance, the course and the tooth abnormalities of cherubism, and also to investigate whether the structure of the lesional tissue varied according to sex, age, family and severity of the disease. The most frequent location of cherubism seems to be a bilateral involvement of the anterior parts of the mandibular rami and molar regions, either with or without involvement of the maxillary tuberosities, combined with aplasia of the second and third molars and malformation of the roots of the mandibular first molars but without pulpal changes. It seems that girls reach the stable period of the disease before boys, at 10 and 14 years of age, respectively. Before the age of 21 the gross and microscopic structure of the lesional tissue is always the same from patient to patient, as well as in the same patient at different ages, and corresponds to the structure of a giant cell granuloma, while in older patients the lesional tissue consists of fibrous tissue.