Thirteen Synchronous Multifocal Calcifying Epithelial Odontogenic Tumors (CEOT): Case Report and Review of the Literature

Abstract

<h3>Introduction</h3> Calcifying epithelial odontogenic tumor (CEOT, Pindborg tumor) is a rare benign, locally aggressive neoplasm of the jaws that accounts for approximately 1% of all odontogenic tumors. The CEOT was first defined by Pindborg in 1955 and has been reported approximately 350 times in the literature; 7 of these reported multiple, synchronous lesions (up to 4). We report the eighth case in an individual with the largest number (by far) of CEOTs reported to date and provide a literature review of multifocal CEOT cases. <h3>Materials and methods</h3> A 30-year-old male presented to the Oral and Maxillofacial Surgery Department of the West Virginia University School of Dentistry (WVU SoD) to extract multiple impacted teeth previous to constructing a complete denture. <h3>Results</h3> A pantograph showed 15 impacted teeth, almost all associated with well-demarcated cyst-like radiolucencies, some with small, ill-defined radiopaque flecks. Microscopically the lesions showed sheets of strands of polygonal epithelial cells with eosinophilic cytoplasm. Spread throughout the epithelium and connective tissue were small spherical amorphous pale purplish calcifications. Each lesion was similar and consistent with a diagnosis of CEOT. <h3>Conclusion</h3> We report a patient with 13 independent CEOTs scattered throughout all quadrants, suggesting genetic mutation of a tumor suppressor gene, such as the PTCH1 gene, which has been previously identified in patients with solitary CEOTs. This is the largest number of Pindborg tumors or any other type of odontogenic tumors, yet reported in a single individual.