Osteosarcoma: A comprehensive review of model systems and experimental therapies.

Osteosarcoma (OSA) is a highly malignant bone tumor for which more than 50% of patients have or will develop metastatic disease, resulting in an abysmal 5-year survival rate of <29%. Despite the advances in science and medicine, the etiology of OSA remains unclear. Similarly, the standard of care (surgery and chemotherapy) has changed little in the past 5 decades. This stagnation in treatment options is in part due to inadequate preclinical models for OSA; many of these models are oversimplified and do not account for the complexities of patient disease. Further, current treatments are harsh and invasive (e.g. high dose chemotherapy and potential limb removal) leading to a reduction in a patient's quality of life (e.g. hearing loss, infertility, neuropathy), highlighting a need for developing more effective treatment strategies. Many experimental therapies have been tested in the preclinical and preclinical setting, with varying degrees of success. In this review, we will focus on pediatric and adolescent OSA, highlighting current animal models and experimental therapies.