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Small cell osteosarcoma of bone, which was first described in 1979, is an unusual variant of osteosarcoma. Osteoid production by tumor cells is frequently focal or minimal, making the differential diagnosis with other small round cell tumors of bone difficult. Here, we present a rare case of small cell osteosarcoma of the mandible appearing as bony bulging mass in 31-year-old male who has neither tenderness nor paresthesia. Histologically, the tumor contains hypercellular cartilage and abnormal osteoid associated with small round to ovoid malignant cells. Awareness of small cell osteosarcoma should be emphasized because it has worse prognosis than both other small round cell tumor and conventional osteosarcoma.
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