Clear Cell Odontogenic Carcinoma: Report of a Case and Analysis of the Literature

Background: Clear cell odontogenic carcinoma (CCOC), which has been described within the past 2 decades, is a rare odontogenic tumor that tends to occur in the mandible of older adults, with a predilection for women. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases.

Objectives: To report the clinicopathologic findings and follow-up of a case of CCOC and to review the literature.

Design: Case report and literature review.

Main Outcome Measurements: Findings from histologic analysis, immunohistochemistry, and electron microscopy.

Results: An 81-year-old woman experienced 3 locoregional recurrences within 21 months of initial therapy. She is presently disease free, 4.5 years after initial resection, having received multiple resections and adjuvant radiotherapy.

Conclusions: The diagnosis of CCOC must be considered in the differential diagnosis of jaw tumors with a clear cell component. For these tumors, resection with negative margins is the treatment of choice because more conservative surgery (eg, curettage) inevitably results in recurrence and/or metastasis. Adjuvant radiotherapy is a rational option for tumors that have eroded cortex.