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Soft Tissue Sarcoma, Version 2.2018, NCCN Clinical Practice Guidelines in Oncology

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.

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Soft Tissue Sarcoma, Version 2.2016, NCCN Clinical Practice Guidelines in Oncology

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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NCCN Task Force Report: Management of Patients with Gastrointestinal Stromal Tumor (GIST)—Update of the NCCN Clinical Practice Guidelines

George D. Demetri, Robert S. Benjamin, Charles D. Blanke, Jean-Yves Blay, Paolo Casali, Haesun Choi, Christopher L. Corless, Maria Debiec-Rychter, Ronald P. DeMatteo, David S. Ettinger, George A. Fisher, Christopher D. M. Fletcher, Alessandro Gronchi, Peter Hohenberger, Miranda Hughes, Heikki Joensuu, Ian Judson, Axel Le Cesne, Robert G. Maki, Michael Morse, Alberto S. Pappo, Peter W. T. Pisters, Chandrajit P. Raut, Peter Reichardt, Douglas S. Tyler, Annick D. Van den Abbeele, Margaret von Mehren, Jeffrey D. Wayne, and John Zalcberg

The NCCN Soft Tissue Sarcoma Guidelines include a subsection about treatment recommendations for gastrointestinal stromal tumors (GISTs). The standard of practice rapidly changed after the introduction of effective molecularly targeted therapy (such as imatinib and sunitinib) for GIST. Because of these changes, NCCN organized a multidisciplinary panel composed of experts in the fields of medical oncology, molecular diagnostics, pathology, radiation oncology, and surgery to discuss the optimal approach for the care of patients with GIST at all stages of the disease. The GIST Task Force is composed of NCCN faculty and other key experts from the United States, Europe, and Australia. The Task Force met for the first time in October 2003 and again in December 2006 with the purpose of expanding on the existing NCCN guidelines for gastrointestinal sarcomas and identifying areas of future research to optimize our understanding and treatment of GIST. (JNCCN 2007;5[Suppl 2]:S1–S29)

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Soft Tissue Sarcoma, Version 2.2012

Featured Updates to the NCCN Guidelines

Margaret von Mehren, Robert S. Benjamin, Marilyn M. Bui, Ephraim S. Casper, Ernest U. Conrad III, Thomas F. DeLaney, Kristen N. Ganjoo, Suzanne George, Ricardo Gonzalez, Martin J. Heslin, John M. Kane III, Joel Mayerson, Sean V. McGarry, Christian Meyer, Richard J. O'Donnell, Benjamin Paz, John D. Pfeifer, Raphael E. Pollock, R. Lor Randall, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, Sridhar Shankar, Brian A. Van Tine, Jeffrey Wayne, Hema Sundar, and Nicole R. McMillian

The major changes to the 2012 and 2011 NCCN Guidelines for Soft Tissue Sarcoma pertain to the management of patients with gastrointestinal stromal tumors (GISTs) and desmoid tumors (aggressive fibromatosis). Postoperative imatinib following complete resection for primary GIST with no preoperative imatinib is now included as a category 1 recommendation for patients with intermediate or high risk of recurrence. The panel also reaffirmed the recommendation for preoperative use of imatinib in patients with GISTs that are resectable with negative margins but associated with significant surgical morbidity. Observation was included as an option for patients with resectable desmoid tumors that are small and asymptomatic, not causing morbidity, pain, or functional limitation. Sorafenib is included as an option for systemic therapy for patients with desmoid tumors.

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Soft Tissue Sarcoma, Version 2.2014

Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Ephraim S. Casper, Ernest U. Conrad III, Thomas F. DeLaney, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Joel Mayerson, Sean V. McGarry, Christian Meyer, Richard J. O’Donnell, Alberto S. Pappo, I. Benjamin Paz, John D. Pfeifer, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, Brian A. Van Tine, Jeffrey D. Wayne, Mary Anne Bergman, and Hema Sundar

These NCCN Guidelines Insights highlight the important updates to the NCCN Guidelines for Soft Tissue Sarcoma (STS) specific to the role of radiation therapy in the management of patients with retroperitoneal/intra-abdominal STS. The guidelines have also included recommendations for genetic testing and counseling for patients with a clinical and/or family history of genetic cancer syndromes associated with a predisposition for the development of STS.

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Gastrointestinal Stromal Tumors, Version 2.2014

Margaret von Mehren, R. Lor Randall, Robert S. Benjamin, Sarah Boles, Marilyn M. Bui, Ephraim S. Casper, Ernest U. Conrad III, Thomas F. DeLaney, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, John M. Kane III, Joel Mayerson, Sean V. McGarry, Christian Meyer, Richard J. O’Donnell, Alberto S. Pappo, I. Benjamin Paz, John D. Pfeifer, Richard F. Riedel, Scott Schuetze, Karen D. Schupak, Herbert S. Schwartz, Brian A. Van Tine, Jeffrey D. Wayne, Mary Anne Bergman, and Hema Sundar

Gastrointestinal stromal tumors (GIST) are the most common soft tissue sarcoma of the gastrointestinal tract, resulting most commonly from KIT or platelet-derived growth factor receptor α (PDGFRα)-activating mutations. These NCCN Guideline Insights highlight the important updates to the NCCN Guidelines for Soft Tissue Sarcoma specific to the management of patients with GIST experiencing disease progression while on imatinib and/or sunitinib.

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NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022

Featured Updates to the NCCN Guidelines

Margaret von Mehren, John M. Kane III, Richard F. Riedel, Jason K. Sicklick, Seth M. Pollack, Mark Agulnik, Marilyn M. Bui, Janai Carr-Ascher, Edwin Choy, Mary Connelly, Sarah Dry, Kristen N. Ganjoo, Ricardo J. Gonzalez, Ashley Holder, Jade Homsi, Vicki Keedy, Ciara M. Kelly, Edward Kim, David Liebner, Martin McCarter, Sean V. McGarry, Nathan W. Mesko, Christian Meyer, Alberto S. Pappo, Amanda M. Parkes, Ivy A. Petersen, Matthew Poppe, Scott Schuetze, Jacob Shabason, Matthew B. Spraker, Melissa Zimel, Mary Anne Bergman, Hema Sundar, and Lisa E. Hang

Gastrointestinal stromal tumors (GIST) are the most common type of soft tissue sarcoma that occur throughout the gastrointestinal tract. Most of these tumors are caused by oncogenic activating mutations in the KIT or PDGFRA genes. The NCCN Guidelines for GIST provide recommendations for the diagnosis, evaluation, treatment, and follow-up of patients with these tumors. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including revised systemic therapy options for unresectable, progressive, or metastatic GIST based on mutational status, and updated recommendations for the management of GIST that develop resistance to specific tyrosine kinase inhibitors.

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Soft Tissue Sarcoma, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology

Margaret von Mehren, John M. Kane, Mark Agulnik, Marilyn M. Bui, Janai Carr-Ascher, Edwin Choy, Mary Connelly, Sarah Dry, Kristen N. Ganjoo, Ricardo J. Gonzalez, Ashley Holder, Jade Homsi, Vicki Keedy, Ciara M. Kelly, Edward Kim, David Liebner, Martin McCarter, Sean V. McGarry, Nathan W. Mesko, Christian Meyer, Alberto S. Pappo, Amanda M. Parkes, Ivy A. Petersen, Seth M. Pollack, Matthew Poppe, Richard F. Riedel, Scott Schuetze, Jacob Shabason, Jason K. Sicklick, Matthew B. Spraker, Melissa Zimel, Lisa E. Hang, Hema Sundar, and Mary Anne Bergman

Soft tissue sarcomas (STS) are rare malignancies 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 Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, and treatment of extremity/superficial trunk/head and neck STS, as well as retroperitoneal/intra-abdominal STS, desmoid tumors, and rhabdomyosarcoma. This portion of the NCCN Guidelines discusses general principles for the diagnosis and treatment of retroperitoneal/intra-abdominal STS, outlines treatment recommendations, and reviews the evidence to support the guidelines recommendations.

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NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021

Featured Updates to the NCCN Guidelines

Margaret von Mehren, John M. Kane III, Marilyn M. Bui, Edwin Choy, Mary Connelly, Sarah Dry, Kristen N. Ganjoo, Suzanne George, Ricardo J. Gonzalez, Martin J. Heslin, Jade Homsi, Vicki Keedy, Ciara M. Kelly, Edward Kim, David Liebner, Martin McCarter, Sean V. McGarry, Christian Meyer, Alberto S. Pappo, Amanda M. Parkes, I. Benjamin Paz, Ivy A. Petersen, Matthew Poppe, Richard F. Riedel, Brian Rubin, Scott Schuetze, Jacob Shabason, Jason K. Sicklick, Matthew B. Spraker, Melissa Zimel, Mary Anne Bergman, and Giby V. George

The NCCN Guidelines for Soft Tissue Sarcoma provide recommendations for the diagnosis, evaluation, treatment, and follow-up for patients with soft tissue sarcomas. These NCCN Guidelines Insights summarize the panel discussion behind recent important updates to the guidelines, including the development of a separate and distinct guideline for gastrointestinal stromal tumors (GISTs); reconception of the management of desmoid tumors; inclusion of further recommendations for the diagnosis and management of extremity/body wall, head/neck sarcomas, and retroperitoneal sarcomas; modification and addition of systemic therapy regimens for sarcoma subtypes; and revision of the principles of radiation therapy for soft tissue sarcomas.

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Adolescent and Young Adult (AYA) Oncology, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology

Smita Bhatia, Alberto S. Pappo, Melissa Acquazzino, Wendy A. Allen-Rhoades, Marie Barnett, Scott C. Borinstein, Robert Casey, Sun Choo, Rashmi Chugh, Shira Dinner, Ralph Ermoian, Douglas Fair, Noah Federman, Jeanelle Folbrecht, Shipra Gandhi, Julie Germann, Robert Goldsby, Robert Hayashi, Alex Y. Huang, Mary S. Huang, Linda A. Jacobs, Cathy Lee-Miller, Michael P. Link, John A. Livingston, Maryam Lustberg, Marcio Malogolowkin, Kevin C. Oeffinger, Christine A. Pratilas, Damon Reed, Jodi Skiles, Margaret von Mehren, Nicholas Yeager, Sarah Montgomery, and Lisa Hang

This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on considerations for the comprehensive care of AYA patients with cancer. Compared with older adults with cancer, AYA patients have unique needs regarding treatment, fertility counseling, psychosocial and behavioral issues, and supportive care services. The complete version of the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.