Clear cell odontogenic carcinoma of the mandible

This article describes a case of clear cell odontogenic carcinoma (CCOC) in a 55-year-old woman who presented such a tumor extending from the midline of the mandible to the right first molar. The tumor was surgically excised and has not recurred or metastasized 6 months after surgery. To date, only 33 well-documented cases have been reported (including the present case) in the English literature. Twenty-eight (84.8%) have developed in the mandible and five (15.2%) in the maxilla. Eighteen (54.5%) occurred anterior to the first molar. The vast majority have been diagnosed in patients older than 40 years (30/33), and the mean age at diagnosis is 57.4 years (range: 17-89), with a male/female ratio: 1/2. Recurrences have been described in 17 cases (51.5%) and metastasis in 10 (30.3%). Based on its morphologic, histochemical and immunophenotypic features, CCOC should be distinguished from other primary and metastatic clear cell tumors of the oral and maxillofacial regions.