Clear cell odontogenic carcinoma: evaluation of reported cases

Abstract

Purpose: We present the cases of 4 patients with clear cell odontogenic carcinoma and a contrasting case of a patient with a calcifying epithelial odontogenic tumor from Massachusetts General Hospital. Differential diagnosis, distinguishing characteristics, and data from 32 cases in the literature are also reported. Because this rare tumor behaves aggressively with both regional and distant spread, a better understanding of its biologic behavior is imperative.

Patients and methods: The 4 patients with clear cell odontogenic carcinoma were women and had an average age of 65 years (range, 40 to 77 years). Three tumors were in the posterior mandible and 1 was in the maxilla. Three patients complained of pain; bony expansion and tooth mobility were noted in isolated cases. A comparison is made with other reported cases, and factors associated with recurrence and survival are presented.

Results: All patients were treated with resection, and 1 patient received postoperative radiotherapy. Two patients with positive surgical margins underwent further resection. All patients have been followed an average of 2 years (range, 0.5 to 4 years) and presently have no evident disease.

Conclusions: Recognition of this unusual tumor and differentiating it from other clear cell neoplasms is critical in developing an appropriate treatment plan. Management should include wide en bloc resection and long-term follow-up.