Clear Cell Odontogenic Carcinoma: A Rare Neoplasm of the Maxillary Bone

Clear cell odontogenic carcinoma (CCOC) is an extremely rare neoplasm, with only 74 cases in the English-language literature. It displays a propensity for the mandible, most commonly presenting in the fifth to seventh decades. Histopathologically, CCOC is characterized by sheets and islands of vacuolated and clear cells. The aggressive nature of CCOC was noted in its first description in 1985, although it was not formally classified as malignant by the World Health Organization until 2005. This report describes a case of CCOC presenting atypically in a young patient and at an uncommon site. The authors review the details of this case, outlining management strategies referencing their experience and that described in the other limited cases in the literature.