CLEAR CELL ODONTOGENIC CARCINOMA OF THE MANDIBLE: CASE REPORT. THALITA SANTANA

Clear cell odontogenic carcinoma (CCOC) is an infrequent tumor that presents an aggressive nature among odontogenic tumors. Here, we report a case of CCOC occurring in the mandible of an 82-year-old female patient. The patient presented with increased volume of the right mandible and facial asymmetry for 10 months. Computed tomography revealed an ill-defined and destructive radiolucency in the body and ramus of the mandible. Surgeons suspected ameloblastoma or ameloblastic carcinoma, and an incisional biopsy was performed. Histopathologic examination revealed a proliferation of islands of epithelial cells, presenting predominantly with clear cytoplasm. Intervening stroma was densely hyalinized, and tumor cells were closely related to lymphovascular structures. Immunohistochemistry showed focal positive reactions to cytokeratins 7 and 19. RT-PCR evidenced the fusion transcript EWSR1-ATF1. A diagnosis of CCOC of the mandible was made, and the patient was recommended for surgical therapy; however, the patient died of the disease before treatment.