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Abstract
Keratocystic odontogenic tumor (KCOT) has been identified as a tumor after observation of its biological behavior and genetic abnormalities consistent with neoplastic progression. In 2005, the World Health Organization (WHO) working group considered odontogenic keratocyst (OKC) to be a tumor and recommended the term KCOT, distinguishing the lesion from the orthokeratinizing variant, which is now considered an OKC or orthokeratinized odontogenic cyst. Very rarely, KCOTs can transform into more aggressive lesions such as ameloblastoma and primary intraosseous carcinoma (PIOSCC). In this paper, we present a case of KCOT involving the angle and ramus of the mandible, with histopathologic evidence of ameloblastomatous changes. We also discuss about the evolution of this lesion from a cyst to a tumor along with the latest updates of the entity.
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