Primary intraosseous carcinoma (PIOC) is a rare and challenging jawbone malignancy often linked to odontogenic cysts. With minimal connection to oral mucosa and a low incidence rate, PIOC presents significant diagnostic difficulties, often mimicking other odontogenic lesions. Histogenesis and the correct classification of the lesion remains debated, with theories suggesting origins from odontogenic epithelium or cysts. Chronic inflammation may contribute to malignant transformation, though genetic predispositions could also play a role in the pathogenesis. This review underscores the current knowledge of the lesion with the need for standardized diagnostic markers and an enhanced understanding of PIOC origin to improve diagnostic accuracy and treatment outcomes.
Keywords: Classification; Inflammation; OSCC; Primary intraosseous carcinoma; Prognosis.
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