Multifocal calcifying epithelial odontogenic tumor.

The calcifying epithelial odontogenic tumor (CEOT), or Pindborg tumor, is a rare and benign odontogenic neoplasm that affects the jaw. The most common manifestation of CEOT is a unifocal or localized lesion of the involved jaw, which may appear clinically as a hard tissue swelling and radiographically as a mixed radiolucent-radiopaque mass. In this article, we present a unique case of CEOT affecting multiple sites in the maxilla and mandible of a 51-year-old white man. Though biopsy samples from all involved sites revealed similar histopathologic features consistent with CEOT, the fact that there was a multifocal presentation is an unusual phenomenon for CEOT and has never been reported. Multifocal odontogenic lesions are not typical but have been observed in conditions associated with known genetic mutations. For example, multiple odontogenic keratocysts are the most common feature of the inherited condition known as nevoid basal cell carcinoma syndrome. This case, however, is the first one to demonstrate that there may be a multifocal variant of CEOT that has not been previously recognized.