Clear cell odontogenic carcinoma: a diagnostic and therapeutic dilemma

BACKGROUND: Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring in the anterior region of the mandible in 5(th)–7(th )decades and shows a female preponderance. It is potentially aggressive, capable of frequent recurrences and loco-regional and distant metastases. CASE PRESENTATION: A 45- year- old woman presented with a radiolucent left mandibular swelling associated with loss of teeth. Left cervical lymph nodes were enlarged on palpation. The patient underwent resection of the tumor but consequent to resected margins being positive for tumor cells underwent left hemimandibulectomy with ipsilateral functional neck dissection and was free of recurrence at 8 months follow-up. CONCLUSION: Clear cell odontogenic carcinoma should be considered in the differential diagnosis of jaw tumors with conspicuous clear cell component. Curettage or conservative resection inevitably results in recurrences and/or metastasis and more radical resection is warranted in these tumors, especially when they are large and show soft tissue invasion.