Calcifying Odontogenic Cyst: An Enigma

The calcifying odontogenic cyst (COC), discovered in 1962, is a rare developmental odontogenic cyst clinically present as slow-growing swelling mainly in the anterior portion of the jaws, generally present in the second and sixth decades of life. It accounts for 0.3%–0.8% of odontogenic cysts. COC showed variations in clinical and radiographic features that are not pathognomic, whereas histomorphology forms exist in 3 patterns: benign cystic, solid (neoplastic), and aggressive (malignant) forms. The radiograph shows well-defined radiolucency with irregular masses, while the histopathologic features include a cystic lining with characteristic “Ghost” cells and immunohistochemical reactions positive for various enamel proteins.