Calcifying odontogenic cyst associated with compound odontoma - A rare entity

Calcifying odontogenic cyst (COC) or Gorlin's cyst categorized as a distinct entity by Gorlin et al. in 1962. It is a rare benign developmental cystic lesion that accounts for less than 1% of all odontogenic cysts. It can occur in association with various types of odontogenic tumors such as odontomas. COC is a hybrid lesion of the jaw presenting a manifold variety of clinical behaviors and histopathological characteristics including cystic, solid (neoplastic), and aggressive forms. COC exhibits diversity in terms of its clinical presentations, histopathologic features, and biological behavior. Normally, it presents as asymptomatic, slow developing lesion affecting the maxilla and mandible equally with strong predilection for the anterior segment. Radiographically, these lesions usually present as a unilocular, well-defined radiolucency with radiopaque structures within the lesion, either as irregular calcifications or tooth-like densities. The distinct features of COC as solid or cystic lesions presents with an ameloblastomous component admixed with varying proportions of ghost cells and spherical calcifications. Here, we report an article of calcifying odontogenic cyst associated with compound odontoma in a young patient with lesion in the anterior mandible with review of the various terminologies and classification and histopathology.