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Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Ernest U. Conrad III, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Henry Koon, Joel Mayerson, Martin McCarter, Sean V. McGarry, Christian Meyer, Richard J. O'Donnell, Alberto S. Pappo, I. Benjamin Paz, Ivy A. Petersen, John D. Pfeifer, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, William D. Tap, Jeffrey D. Wayne, Mary Anne Bergman, and Jillian Scavone

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for Soft Tissue Sarcoma (available at NCCN.org) provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumor, desmoid tumors, and rhabdomyosarcoma. This manuscript discusses guiding principles for the diagnosis and staging of STS and evidence for treatment modalities that include surgery, radiation, chemoradiation, chemotherapy, and targeted therapy.

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Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Vicki Keedy, Edward Kim, Henry Koon, Joel Mayerson, Martin McCarter, Sean V. McGarry, Christian Meyer, Zachary S. Morris, Richard J. O'Donnell, Alberto S. Pappo, I. Benjamin Paz, Ivy A. Petersen, John D. Pfeifer, Richard F. Riedel, Bernice Ruo, Scott Schuetze, William D. Tap, Jeffrey D. Wayne, Mary Anne Bergman, and Jillian L. Scavone

Soft tissue sarcomas (STS) are rare solid tumors of mesenchymal cell origin that display a heterogenous mix of clinical and pathologic characteristics. STS can develop from fat, muscle, nerves, blood vessels, and other connective tissues. The evaluation and treatment of patients with STS requires a multidisciplinary team with demonstrated expertise in the management of these tumors. The complete NCCN Guidelines for STS provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as intra-abdominal/retroperitoneal STS, gastrointestinal stromal tumors, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis, staging, and treatment of STS of the extremities, superficial trunk, or head and neck; outlines treatment recommendations by disease stage; and reviews the evidence to support the guidelines recommendations.