An Unusual Variant of a Calcifying Epithelial Odontogenic Tumor in the Maxilla: A Case Report

Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, is a rare benign odontogenic neoplasm that exhibits locally aggressive behavior. Histologically, it is characterized by sheets and nests of polyhedral epithelial cells with eosinophilic or, less frequently, clear cytoplasm. Other hallmark features include extracellular amyloid-like deposits, concentric calcifications known as Liesegang rings, and nuclear pleomorphism without significant mitotic activity. CEOTs are extremely uncommon in the maxilla, but when present in this location, they tend to exhibit more aggressive biological behavior and a higher recurrence rate compared to those in the mandible. We report a rare case of the macrocystic variant of CEOT in a 26-year-old female patient. Histopathological examination of the incisional biopsy revealed epithelial cell proliferation with eosinophilic, clear, and vacuolated cytoplasm interspersed with foci of amyloid-like material. Following diagnosis, complete enucleation of the tumor was performed under local anesthesia. The relevance of the clear cell variant of CEOT lies in its rarity, potential for more aggressive behavior, and diagnostic challenges. While the presence of clear cells in an odontogenic tumor is unusual, it can indicate a higher chance of recurrence and necessitates careful histopathological diagnosis to avoid confusion with other clear cell neoplasms, guiding more aggressive treatment and follow-up.