Peripheral intraosseous carcinoma arising from a recurrent odontogenic keratocyst

Primary intraosseous carcinoma (PIOC) is a distinct clinical and genomic pathology that should be considered when treating aggressive odontogenic cysts refractory to standard treatments. Odontogenic cysts have a very low chance of malignant transformation, with most studies citing an incidence of <0.05%. The pathogenesis of PIOC is multifactorial, with chronic inflammation-induced carcinogenesis being the most cited theory. Early detection is imperative to enhance the patient’s prognosis, with the 5-year overall survival rate of PIOC being 38%. This paper presents a case of PIOC and reviews data on its epidemiology, diagnosis, presentation, and treatment.