Clear cell odontogenic carcinoma of the maxilla: a case report and literature review

BackgroundClear cell odontogenic carcinoma (CCOC) is a rare tumor of odontogenic origin. It was first described as a clinicopathological entity in 1985, and since then, several case reports have been published.MethodsWe present a patient with CCOC treated at the University of Florida and review the pertinent literature.ResultsWe treated a 52-year-old woman with CCOC of the maxilla. She received a maxillectomy and postoperative radiotherapy (RT). She developed pulmonary metastases 2 years after treatment. She remains alive and asymptomatic 7 years after completing treatment and has not required additional therapy.The literature search yielded 48 other case reports. The median age at presentation was 53 years (mean, 55 years), the female-to-male ratio was 2:1, and the most common site of presentation was the mandible. Three patients presented with submandibular lymphadenopathy. Most patients had a surgical resection (35 patients, 71%); the remainder underwent curettage. Six (12%) patients received postoperative RT. Eighty percent of patients who were initially treated with curettage developed a local recurrence. Local and/or regional recurrences occurred in 34% of patients who underwent surgical resection. Six patients developed distant metastatic disease; the most common site of the metastases was the lung. Seven patients died from or with the disease, and 3 are alive with the disease.ConclusionsClear cell odontogenic carcinoma is a rare tumor of the mandible and maxilla that has the propensity for local destruction and the potential for lymphatic and hematogenous metastases. We recommend radical excision as the primary treatment and that adjuvant RT be added for those who have close or positive margins as well as those with lymph node metastases.