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Primary intraosseous carcinoma (PIOC) is a rare neoplasm occurring in the jaw bones, especially in younger patients which is locally aggressive and has a poor prognosis. It is believed to arise from the odontogenic epithelium. It is called as PIOC because it arises de novo which makes it primary and it develops centrally within the bone hence called as intraosseous. The early lesions are asymptomatic or a painful swelling is seen. To diagnose PIOC, basic criteria have been proposed. (1) Absence of ulcer in the oral mucosa overlying the tumor, (2) Absence of another primary tumor at the time of diagnosis and for at least 6 months during the follow-up, and (3) Histological evidence of squamous cell carcinoma. Till date, around 60 cases of PIOC are reported. Being rare, we would like to discuss the review of literature and a case report on PIOC.
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