Ameloblastomatous Calcifying Odontogenic Cyst: A Rare Entity

INTRODUCTION: Ameloblastomatous calcifying odontogenic cyst (COC) is an extremely rare histopathologic variant of COC, an odontogenic cyst of the jaws. It needs to be differentiated from closely associated variant ameloblastoma ex COC that is entitled to a more aggressive form of surgical management. AIM: The aim of this paper is to present a case of ameloblastomatous COC of the right mandibular angle region with review of literature describing this rare entity. The paper also highlights the requirement of including this lesion in differential diagnosis of various jaw lesions. MATERIALS AND METHODS: Google search, Wikipedia, ScienceDirect, MEDLINE, the Cochrane library, and PubMed were used extensively to search and collect all reported cases of ameloblastomatous COC using keywords such as ameloblastomatous COC, COC, maxilla, mandible, and gorlin cyst ameloblastic proliferation. RESULTS: To the best of our knowledge, a total of twenty one cases of ameloblastomatous COC have been reported in the literature in the maxilla-mandibular region and we represent the twenty second case in a 20-year-old female patient along with the review. CONCLUSION: Ameloblastomatous COC – a subtype of COC needs to be differentiated from true ameloblastoma arising from COC as it warrants a conservative form of surgical management unlike other neoplastic variants of COC. Owing to scarcity of data of this lesion in literature, more reporting of such cases is required to shed light on its behavior.