Osteosarcomas are highly malignant bone tumours characterised by the presence of malignant mesenchymal cells producing osteoid or immature bone. Though rapidly growing bones are commonly affected like metaphysis in the femur, rare cases of Osteosarcoma of jaw (JOS) too occur; with an incidence of approximately 0.7 per million. The diagnosis involves clinico-radiological and histological correlation. A 14-year old male presented with the chief complaints of swelling and pain in his lower jaw on left side for 15 days and was given a provisional diagnosis of neoplasia of left body of mandible on radiography which was confirmed as osteosarcoma in histopathology. The case presented here highlight the importance of histopathology in diagnosing the lesion. Considering its rarity we present the case and attempt to review the literature associated with this lesion. Osteosarcoma especially of jaw requires a high degree of suspicion and need to be diagnosed and treated promptly to prevent further complications as rapidly growing tumour could predispose to bony outgrowth, facial deformity, airway compromise and metastasis. Key message: Jaw osteosarcoma is a rare entity requiring clinico-radiological and histopathological corelation for early diagnosis.With a clinically distinct profile as compared to its extremity counterpart, early histomorpholgical diagnosis and management with surgery, chemotherapy and radiotherapy can lead to better outcome. Keywords: Bone malignant tumour, Histopathology, Jaw osteosarcoma, Mandible, Osteoblastic.
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