Calcifying Epithelial Odontogenic Cyst of Maxilla: A Clinical and Histological Rarity with Literature Review

A cyst is defined as a pathological cavity which may or may not have an epithelial lining and which has a fluid, semi-fluid, or gaseous contents and is not formed by the accumulation of pus. The calcifying epithelial odontogenic cyst (CEOC) was first reported by Gorlin et al. in 1962. At that time, it was classified as a cyst related to the odontogenic apparatus. It was later renamed as calcifying cystic odontogenic tumor (CCOT) in the World Health Organization classification devised in 2005 due to its histological complexity, morphological diversity, and aggressive proliferation. CCOT was later recognized by numerous names including Gorlin cyst, calcifying ghost cell odontogenic cyst and/or dentogenic ghost cell tumor. It has a peak incidence during the second and third decades of life and does not demonstrate any gender predilection. Radiographically, CEOC may appear as a unilocular or multilocular radiolucent lesion with either well-circumscribed or poorly-defined margins and may also be observed in association with unerupted teeth. Calcification is an important radiographic feature for the interpretation of CEOC/CCOT. The typical histopathological features of CEOC include a fibrous wall and lining of the odontogenic epithelium with either columnar or cuboidal basal cells resembling ameloblasts. The treatment of choice for CEOC is conservative surgical enucleation; however, recurrence is also not found to be uncommon. Herein, we are reporting a case of the same in a 21-year-old female which was a great dilemma during the diagnostic workup.