Central mucoepidermoid carcinoma: a clinicopathologic and immunohistochemical study of 39 Chinese patients.

Central mucoepidermoid carcinoma (MEC) is a rare neoplasm arising intraosseously in the jaws. To clarify the clinicopathologic profile and pathogenesis of central MEC, clinicopathologic findings and follow-up data of 39 cases were collected and analyzed. There were 16 male and 23 female patients (median age, 43 y). Sixteen cases affected the maxilla, and 23 occurred in the mandible. Radiographically, most cases (32 of 39) showed a unilocular or multilocular radiolucency with bone destruction, and 7 were found with scattered calcification. The margins of the lesions were ill defined or diffused in 14 cases and relatively well defined in 25 cases. Most cases (26 of 39) were classified as low-grade MECs, whereas 13 were moderate-to-high grade. Follow-up data were available for 35 patients with a median period of 36 months. All cases were found to be primary; local recurrence occurred in 8 cases, most (75.0%) of which were low-grade tumors. Four cases showed regional lymph node metastasis, and 1 developed distant metastasis. Of 11 cases with a clinical history of the jaw cyst, 8 initially showed a typical odontogenic cyst with local MEC-like proliferation. In summary, the most likely pathogenesis of central MEC is neoplastic transformation of the epithelial lining of an odontogenic cyst, diagnosis of which should be based on clinical, radiographic, and histopathologic findings. The immunohistochemical profile of keratins is helpful in differential diagnosis. Radical surgery is the treatment of choice, whereas the role of radiotherapy or chemotherapy is still controversial, and careful long-term follow-up is necessary.