Ameloblastomatous calcifying odontogenic cyst: A rare histologic variant

Calcifying odontogenic cyst (COC) is an uncommon developmental odontogenic cyst first described by Gorlin in 1962. It is considered as extremely rare and accounts for only 1% of jaw cysts reported. Because of its diverse histopathology, there has always been confusion about its nature as a cyst, neoplasm or hamartoma. Several subclassifications have been proposed. Here, we present a case of calcifying odontogenic cyst with ameloblastic proliferation - an extremely rare histologic variant. The classical histologic features of the lining epithelium in the form of cords and presence of characteristic ghost cells were seen along with ameloblastomatous proliferations. Ameloblastomatous COC microscopically resembles unicystic ameloblastoma except for the ghost cells and calcifications within the proliferative epithelium. The nature of the COC is controversial. The case is presented here for its rarity, and difference between ameloblastomatous COC and ameloblastoma ex COC has been emphasized.