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Osteosarcoma is a malignant bone primary tumour characterized for osteoid synthesis produced by malignant osteoblasts. Evidence of osteoid formation by neoplastic cells is considered an essential criterion for diagnosis and microscopic evidence of neoplastic bone, even if minimal, confirms the diagnosis. The jawbone is considered to be the most common site in the head and neck region. Maxillary swelling and pain associated tooth mobility is the most common clinical manifestation and radiographically presented as a mixed osteolytic lesion, or osteoblastic. Histopathologically it is characterized by the formation of osteoid. This article presents the case of a woman of 45 years old, diagnosed with low-grade fibroblast osteosarcoma. Given the rarity of the tumor, we present a case report and literature review, which represents an important contribution to a better understanding of the maxillary osteosarcomas is made.
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