Abstract
Background: Odontogenic tumors arising from extra-alveolar sites are extremerly rare. Dentinogenic ghost cell tumor (DGCT) is an uncommon odontogenic neoplasm characterized by CTNNB1 mutation, ghost cell appearance, and dentinoid-like calcification. We present a case of an ectopic DGCT arising from a calcifying odontogenic cyst in the floor of the mouth. Case presentation: A 72-year-old man presented with a painless sublingual swelling. Imaging revealed a multi-lobulated, solid-cyst mass on the floor of the mouth. Cytology showed folded epithelial clusters composed of basaloid cells, keratinized material, and dentinoid matrix. Histology also revealed a multi-cystic, cribriform to solid nest. Immunohistochemically, CK19, CK5/6, bcl-2, and p63 were diffuse positive. CTNTTB1 mutation was detected, leading to the final diagnosis of an ectopic DGCT. There was no recurrence during a 6-month follow-up. Conclusion: This is the first report to comprehensively describe the clinicopathological features of an ectopic DGCT of odontogenic origin, developing similarly to that of a true odontogenic DGCT. Accurate diagnosis of this rare entity is necessary to avoid overtreatment.