Cherubism: review of the literature

Abstract

This review aimed at reporting on cherubism, a hereditary form of benign fibro-osseous lesion thought to be inherited as an autosomal dominant trait with a great deal of variability in its expression. Bone lesions begin in early childhood as multiple litic areas of characteristic expansile slow-growing development that lead to severe facial deformity. As the patient reaches puberty the lesions stabilize but the disfigured appearance (characteristic cherubistic facies) jeopardizes the patient's social relationship. Considering the critical role played by image-based examinations where pathological anatomy is inconclusive, we have proposed, as part of the diagnostic approach, taking first the panoramic radiograph and finally the CT as definitive examination. The appropriateness of including the craniofacial fibrous dysplasia in the differential diagnosis is discussed, with emphasis on the clinical and image differences between the conditions.