Osteosarcoma of the jaws is a relative rare malignant bone tumor. Like, its counterpart in the long bones, osteosarcoma affecting the head and neck region shows distinct yet diverse clinical, histologic and prognostic characteristics. Its diagnosis is a challenge to histopathologists and is especially important in early stages to improve its prognosis. In the initial phase, it may present as nondescript swelling with an indolent growth rate, only to become overtly aggressive and malignant towards the later phase of the disease. This article reports on a case of an advanced osteosarcoma of the maxilla in a 42 year old woman who came for the evaluation of a swelling. The case was submitted for surgical intervention and was followed by one recurrence till the time of reporting. This case illustrates that immunohistochemical staining of Vimentin, S-100 and CD 68 markers are useful to confirm the histologic diagnosis of osteosarcoma, along with radiographic evaluation using CT scan and 3D imaging.
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