Sarcomas

Malignant bone tumors (osteosarcoma, Ewing sarcoma) and soft-tissue sarcomas (rhabdomyosarcoma, nonrhabdomyosarcoma) account for approximately 14% of childhood malignancies. Successful treatment of patients with sarcoma depends on a multidisciplinary approach to therapy, including oncology, surgery, radiation oncology, radiology, pathology, and physiatry. By combining systemic treatment with chemotherapy and primary tumor control using surgery and/or radiation, survival rates for localized disease range from 70% to 75%. However, children with metastatic or recurrent disease continue to have dismal outcomes. A better understanding of the biology underlying both bone and soft-tissue sarcomas is required to further improve outcomes for children with these tumors.

Key points

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Osteosarcoma and Ewing sarcoma are the 2 most common malignant bone tumors of pediatrics, with peak incidence during the adolescent years.
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Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood, with a bimodal age distribution of 2 to 6 years and 15 to 19 years of age.
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Sarcomas are best treated with multimodality treatment that often includes chemotherapy, surgery, and/or radiation therapy.
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Children with localized sarcoma fare well with multimodality treatment, whereas those with metastatic or recurrent disease have poor outcomes.
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Improved molecular understanding of these diseases has led to the possibility of using new molecular targeted agents in the setting of clinical trials.