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Odontogenic tumors (OTs) are rare lesions, exclusive of the jaws, that are derived from epithelial and/or ectomesenchymal elements of the tooth-forming apparatus. Their biological behavior is heterogeneous, including hamartomatous tissue proliferation, benign nonaggressive and aggressive neoplasms, and malignant tumors with metastatic capacity. The aim of this study was to describe the relative frequency of odontogenic tumors in a Brazilian population. In addition, a review of the literature identified studies on odontogenic tumors that follow the 2005 World Health Organization. A total of 376 cases of odontogenic tumors from an oral pathology service were reviewed about age, gender, anatomic site and histologic diagnosis. Keratocystic odontogenic tumors (31.6%) were the most common, followed by ameloblastoma (28.5%), and odontoma (22.6%). The mean age was 32.2 years, and more than half the patients (52.1%) were in the second and third decades of life. The male to female ratio was 1:1.37, with a maxilla to mandible ratio of 1:2.08. The variation in relative frequency of tumors observed among the several series, including the present study, is probably due in part to cultural differences between geographic areas but also to the study design.
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