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.
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
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.
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.
Peter F. Coccia, Alberto S. Pappo, Lynda Beaupin, Virginia F. Borges, Scott C. Borinstein, Rashmi Chugh, Shira Dinner, Jeanelle Folbrecht, A. Lindsay Frazier, Robert Goldsby, Alexandra Gubin, Robert Hayashi, Mary S. Huang, Michael P. Link, John A. Livingston, Yousif Matloub, Frederick Millard, Kevin C. Oeffinger, Diane Puccetti, Damon Reed, Steven Robinson, Abby R. Rosenberg, Tara Sanft, Holly L. Spraker-Perlman, Margaret von Mehren, Daniel S. Wechsler, Kimberly F. Whelan, Nicholas Yeager, Lisa A. Gurski and Dorothy A. Shead
This selection from the NCCN Guidelines for Adolescent and Young Adult (AYA) Oncology focuses on treatment and management considerations for 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 AYA Oncology addresses additional aspects of caring for AYA patients, including risk factors, screening, diagnosis, and survivorship.