One case of a newly described, rare odontogenic tumor is reported. A literature review of 19 cases lends support to the odontogenic origin and metastatic capability, both regionally and distantly, of the clear cell odontogenic tumor. We conclude that this tumor is malignant, with a tendency to recur locally after surgery. It requires an aggressive surgical approach and should be referred to as a clear cell odontogenic carcinoma. In our patient we saw a clear cell odontogenic carcinoma and a squamous cell carcinoma, both with regional lymph node metastasis. The histological differentiation of this type of tumor from other clear cell tumors is difficult.
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