Four cases of either combined occurrence of ameloblastoma and odontogenic keratocyst or a rare keratinising variant of ameloblastoma are presented. The cardinal histomorphologic characteristics are simultaneous occurrence of ameloblastomatous epithelial islands with central keratinisation and multiple keratinising cysts. Immunohistochemically the tumour elements were keratin positive and occasionally S-100 protein and desmin positive. Major differential diagnosis of these neoplasms are discussed.
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