Clear cell odontogenic carcinoma an enigmatic diagnostic challenge

Clear cell odontogenic carcinoma (CCOC) is an extremely rare odontogenic carcinoma with few cases reported in literature. In 2005, the WHO reclassified CCOC as a malignant odontogenic neoplasm, exhibiting an aggressive growth pattern with a propensity for regional, nodal, and distant metastasis. Being characterized histopathologically by sheets and islands of clear and vacuolated cells, it poses a diagnostic challenge to the clinician and pathologist to distinguish CCOC from other perplexing clear cell entities and requires immunohistochemical analysis. Here, we report a case of CCOC in a 50-year-old woman who presented with a solitary, diffuse, and tender swelling in the posterior aspect of the mandible for 1½ months. Wide local excision was performed for the case. The present case may aid to delineate its biological behavior along with shedding light on its histopathological and immunohistochemical character, thus contributing to the literature.