Clear cell odontogenic carcinoma in maxilla: A rare case report

A rare and aggressive form of tumor with a female predilection and more seen with mandible is the clear cell odontogenic carcinoma (CCOC). The World Health Organization categorized it as a malignant neoplasm in 2005. This type of tumor is characterized by local recurrences as well as distant metastasis apart from being highly aggressive in nature. Due to the presence of clear cells, it is a diagnostic challenge to the pathologist. Only about 84 cases of maxillary variant have been reported in the literature. Here, we present a case report of a 31-year-old male with CCOC in the maxilla from the pathological and diagnostic point of view.