Experimental models

To understand the pathophysiologic mechanisms of human bone and soft tissue sarcomas, and develop interventions to treat them, it became necessary to study sarcomas outside of the human body and deconstruct the events leading to full tumor formation. This has taken the form of human tumor-derived cell lines grown in culture, human tumor-derived cell lines and primary human tumors grown as xenografts in immunocompromised laboratory mice, genetically defined sarcoma cell lines, genetically engineered mouse models, and novel models of sarcoma arising spontaneously in domesticated animals or developed in lower organisms including zebrafish and fruit flies. This chapter reviews the uses of these approaches in understanding bone and soft tissue sarcomas and complements other recent sarcoma model reviews (O’Brien et al. 2012; Kashi et al. 2015).