Appendicular osteosarcoma (OS) is a primary mesenchymal tumor arising from malignantly transformed osteoblasts. In people, OS is the most common nonhematopoietic, primary skeletal neoplasm diagnosed in adolescents and is the second leading cause of cancer-related fatalities within this age group. Despite aggressive therapeutic management, including limb-sparing surgeries and dose-intense systemic chemotherapies, 30-40% of patients will experience progressive metastatic disease within 5 years of diagnosis. In order to reduce the fatality rate associated with recurrent or metastatic OS, a more thorough understanding of OS pathogenesis and biology is required. Towards this pursuit, comparative animal models of OS have been developed and are actively being studied to expand our fundamental understanding of OS. It is anticipated that specific animal models of OS, which most accurately recapitulate the natural disease process in people, will be most useful for advancing our understanding of OS biology, and will facilitate the discovery of disease pathogenesis and the identification of novel therapeutic strategies for managing this lethal metastatic bone sarcoma.
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