Orthokeratinized Odontogenic Cyst: Insights From a Case Series With a Review of Literature

Orthokeratinized odontogenic cyst (OOC) is an uncommon developmental cyst of odontogenic origin that was considered a variant of the odontogenic keratocyst (OKC). However, extensive research has demonstrated that OOC exhibits distinctive clinical, radiological, and histopathological features, warranting its classification as a distinct pathological entity. Histologically, OOC presents a thin, uniform epithelial lining with a prominent orthokeratinized surface and a distinct granular cell layer, features that clearly differentiate it from the parakeratinized and corrugated epithelial lining typically observed in OKC. OOC shows a slow-growing, less aggressive behavior and minimal recurrence potential. The preferred treatment is surgical enucleation, and the prognosis is excellent, with recurrence rates reported to be below 2%, in sharp contrast to the 8%-25% observed in OKC following similar management. This case series highlights six OOC cases, elaborating on their clinical, radiographic, and histopathological features to enhance clinicians' understanding of this rare odontogenic cyst.