Craniopharyngiomas and odontogenic tumors mimic normal odontogenesis and share genetic mutations, histopathologic features, and molecular pathways activation.

Odontogenic tumors bear some histopathologic and molecular resemblance to craniopharyngiomas. Specifically, adamantinomatous craniopharyngioma shares morphologic features and CTNNB1 (the gene encoding β-catenin) mutations with calcifying odontogenic cyst, whereas papillary craniopharyngioma and ameloblastoma are driven by BRAF mutations. Recently, important similarities between adamantinomatous craniopharyngioma and the cell signaling pathways involved in tooth formation have been described. Here, we expand the interpretation of these data in the context of odontogenic tumors. We discuss some morphologic and molecular features that are shared by tumors from these 2 distinct sites (i.e., craniopharyngiomas and odontogenic tumors). Current conservative surgical treatment is effective in most cases of benign odontogenic tumors, but in the future, the understanding of the molecular pathogenesis could impact the treatment of aggressive and/or malignant cases.