Clear cell odontogenic carcinoma of the mandible and temporomandibuar joint with cervical lymph nodal metastasis

Clear cell odontogenic carcinoma (CCOC) is a rare aggressive tumor that has the ability to invade locally as well as cause regional and distant metastasis. The etiology of this neoplasm remains poorly understood and the diagnosis of CCOC is done by exclusion of other clear cell tumors. To date, approximately 75 cases of CCOC have been described in the English literature, all involving a single jaw. The majority of cases have been reported to arise from the mandible, the maxilla is less frequently involved, and no case had involvement of the temporomandibuar joint. Lymph node metastasis at initial presentation is reportedly rare (<10%). We describe possibly the first case of CCOC in a 50-year-old woman with involvement of the mandible and the temporomandibuar joint along with cervical lymph nodal metastasis. We share our experiences and challenges in the management of this unusual tumor.