* Osteosarcoma, while rare, is the most common primary bone sarcoma and accounts for up to 10% of all new pediatric cancer diagnoses annually in the United States. * Most commonly, osteosarcoma affects the distal part of the femur and occurs as a high-grade intramedullary (conventional) subtype. * Patients with osteosarcoma are managed with a multidisciplinary team approach. Often, an orthopaedic oncologist initiates the workup after making a presumptive diagnosis on the basis of classic clinical and radiographic findings. Advanced imaging and a tissue biopsy are performed to evaluate the extent of disease and to histologically confirm the diagnosis. Musculoskeletal radiologists and pathologists are key team members who evaluate the imaging and tissue samples to make a definitive diagnosis, establish a prognosis, and help the clinicians to develop a treatment plan. Medical/pediatric oncologists are essential team members who provide the appropriate neoadjuvant and adjuvant chemotherapy treatment and assist with long-term surveillance to monitor for local or distant relapse. Orthopaedic oncologists develop and execute a plan for resection of the tumor followed by appropriate reconstruction. * The current standard of care for distal femoral osteosarcoma is neoadjuvant chemotherapy followed by limb salvage for the surgically resectable tumor, reconstruction of the bone and soft-tissue defect, and adjuvant chemotherapy. * The survival rate for patients with isolated osteosarcoma is approximately 70% and has not substantially improved in >25 years.
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Stitzlein et al. (2017) studied this question.
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