A rare multilocular cystic disease of the mandible: Cherubism [Mandibulanin nadir multiloküler kistik hastaliği: Şerubizm]

Abstract

Cherubism is a rare skeletal dysplasia located to the maxilla and mandible which is characterized by bilateral, symmetric, fibro-osseous and multilocular cystic lesions. In most of the cases, dominant mutations are detected in the SH3BP2 gene on chromosome 4p. While affected children seem to be clinically normal at birth, whose jaws start swelling and enlargement between 2 and 7 years of age. Full round cheek and upward tilting of the eyes consist of typical appearance of children. Apart from various cystic lesions of mandible, cherubism is a tumor which regresses spontaneously, ossifies over time and has a likelihood for leaving unrecognizable remnants after years, although radiologically, extensive bone involvement appears. Presented here was a patient who suffered from this rare disease and his clinical findings.