Primordial odontogenic tumor: a case report of a rare entity in the Saudi population.
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INTRODUCTION: Primordial odontogenic tumor (POT) is a rare, benign, mixed epithelial–mesenchymal neoplasm, histologically characterized by spindle- to stellate-shaped cells in a fibromyxoid stroma resembling dental papilla, bordered by columnar epithelium. It occurs mainly in children and adolescents, often in the posterior mandible, and radiographically presents as a well-defined radiolucency associated with unerupted teeth. CASE PRESENTATION: An 18-year-old male presented with asymptomatic swelling of the right mandible. Imaging revealed a well-demarcated unilocular radiolucency extending to the ascending ramus with cortical thinning and perforation. Under a provisional diagnosis of ameloblastoma, right mandibulectomy with plate reconstruction was performed. Histology showed spindle and stellate cells in a delicate collagenous stroma lined by columnar epithelium with reverse nuclear polarity and areas of solid and cystic epithelial proliferation, confirming POT. CONCLUSION: We report the first case of POT in Saudi Arabia, managed surgically with no recurrence at seven-year follow-up. This case highlights the diagnostic challenges of POT and the importance of continued case documentation to guide recognition and management.