Osteosarcoma/Ewing Sarcoma

Based on strong research evidence (level A) in the form of phase 3 clinical trials and clinical observation, most cases of pediatric bone cancers are believed to spontaneously develop. Relatively rare genetic conditions, such as Li-Fraumeni, Bloom, and Rothmund-Thomson syndromes, can predispose to the development of osteosarcoma. • Based on strong research evidence (level A) in the form of phase 3 clinical trials and clinical observation, bone sarcomas may present with a palpable or visible mass that may either be painful or painless. It may be first recognized as a mass on imaging acquired to investigate a complaint of pain or after an injury. Laboratory tests are of no specific diagnostic utility; although alkaline phosphatase and/or lactate dehydrogenase levels may be elevated in response to bony destruction, they are not specific markers. • Based on strong research evidence (level A) in the form of phase 3 clinical trials and clinical observation, evaluation of a suspected bone sarcoma involves dedicated imaging of the lesion, imaging to assess for metastatic disease, and biopsy for pathologic diagnosis.Based on strong research evidence (level A) in the form of phase III clinical trials, neoadjuvant chemotherapy, followed by local control surgery and adjuvant chemotherapy with methotrexate, cisplatin, and doxorubicin is the standard of care for localized osteosarcoma. • Based on strong research evidence (level A) in the form of phase III clinical trials, neoadjuvant chemotherapy with an emphasis on surgery to remove evidence of localized and metastatic disease where possible is the standard of care for metastatic osteosarcoma. Prognosis remains poor for those with metastatic osteosarcoma. • Based on strong research evidence (level A) in the form of phase III clinical trials, the standard treatment approach for localized Ewing sarcoma is neoadjuvant chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide alternating every 2 weeks followed by local control with either radiotherapy or surgery and adjuvant chemotherapy. • Based on strong research evidence (level A) in the form of phase III clinical trials, metastatic disease is the most important predictor of prognosis in Ewing sarcoma. Other factors that have been shown to lead to a poorer prognosis include older age at diagnosis, tumor volume larger than 200 mL or largest diameter greater than 8 cm, presence and number of bone lesions, bone marrow involvement, and tumors located in the axial skeleton. • Based on strong research evidence (level A) in the form of clinical research and clinical guidelines, monitoring for long-term late effects related to chemotherapy and radiotherapy exposures is essential in the management of osteosarcoma and Ewing sarcoma.