Abstract: Objective: To investigate the clinicoradiological features of clear cell odontogenic carcinoma (CCOC) in the jaw, summarize its imaging characteristics, and improve the differential diagnosis and understanding of this rare disease. Methods: A retrospective analysis was performed on the clinical and CT imaging features of six cases of CCOC confirmed by histopathological examination. Results: The six CCOC patients had a mean age of 59.6 years and a male-to-female ratio of 2∶1, with disease duration ranging from 2 months to 5 years. Lesions predominantly occurred in the posterior jaw. Characteristic imaging findings included bone destruction with jaw expansion (more pronounced in the mandible and milder in the maxilla), locally aggressive margins, and soft tissue mass formation. Mandibular lesions primarily presented as expansive bone destruction, whereas maxillary lesions exhibited moth-eaten or erosive bone destruction. Fluorescence in situ hybridization was performed in three cases, revealing Ewing sarcoma breakpoint region (EWSR1) gene rearrangement in two cases and EWSR1/Activating transcription factor 1 (ATF1) gene fusion in one case. Conclusion: Mandibular CCOC typically presents as expansive bone destruction with locally aggressive imaging features, requiring differentiation from other locally aggressive lesions. Maxillary CCOC shows erosive bone destruction with soft tissue mass formation, exhibiting imaging signs consistent with low-grade malignancy, and must be distinguished from other malignant tumors of the jaw.

Key words: jaw, clear cell odontogenic carcinoma, clinical manifestation, imaging features