NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021

Featured Updates to the NCCN Guidelines

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  • 1 Fox Chase Cancer Center;
  • | 2 Roswell Park Comprehensive Cancer Center;
  • | 3 Moffitt Cancer Center;
  • | 4 Massachusetts General Hospital Cancer Center;
  • | 5 The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute;
  • | 6 UCLA Jonsson Comprehensive Cancer Center;
  • | 7 Stanford Cancer Institute;
  • | 8 Dana-Farber/Brigham and Women’s Cancer Center;
  • | 9 O'Neal Comprehensive Cancer Center at UAB;
  • | 10 UT Southwestern Simmons Comprehensive Cancer Center;
  • | 11 Vanderbilt-Ingram Cancer Center;
  • | 12 Memorial Sloan Kettering Cancer Center;
  • | 13 Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance;
  • | 14 University of Colorado Cancer Center;
  • | 15 Fred & Pamela Buffett Cancer Center;
  • | 16 The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins;
  • | 17 St. Jude Children’s Research Hospital/The University of Tennessee Health Science Center;
  • | 18 University of Wisconsin Carbone Cancer Center;
  • | 19 City of Hope National Medical Center;
  • | 20 Mayo Clinic Cancer Center;
  • | 21 Huntsman Cancer Institute at the University of Utah;
  • | 22 Duke Cancer Institute;
  • | 23 Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute;
  • | 24 University of Michigan Rogel Cancer Center;
  • | 25 Abramson Cancer Center at the University of Pennsylvania;
  • | 26 UC San Diego Moores Cancer Center;
  • | 27 Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine;
  • | 28 UCSF Helen Diller Family Comprehensive Cancer Center; and
  • | 29 National Comprehensive Cancer Network.

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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    Gounder MM, Thomas DM, Tap WD. Locally aggressive connective tissue tumors. J Clin Oncol 2018;36:202209.

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    Colombo C, Miceli R, Lazar AJ, et al.. CTNNB1 45F mutation is a molecular prognosticator of increased postoperative primary desmoid tumor recurrence: an independent, multicenter validation study. Cancer 2013;119:36963702.

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    Burtenshaw SM, Cannell AJ, McAlister ED, et al.. Toward observation as first-line management in abdominal desmoid tumors. Ann Surg Oncol 2016;23:22122219.

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    Penel N, Le Cesne A, Bonvalot S, et al.. Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: a nationwide prospective cohort from the French Sarcoma Group. Eur J Cancer 2017;83:125131.

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    Choi SH, Yoon HI, Kim SH, et al.. Optimal radiotherapy strategy for primary or recurrent fibromatosis and long-term results. PLoS One 2018;13:e0198134.

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    Bates JE, Morris CG, Iovino NM, et al.. Radiation therapy for aggressive fibromatosis: the association between local control and age. Int J Radiat Oncol Biol Phys 2018;100:9971003.

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    Bishop AJ, Zarzour MA, Ratan R, et al.. Long-term outcomes for patients with desmoid fibromatosis treated with radiation therapy: a 10-year update and re-evaluation of the role of radiation therapy for younger patients. Int J Radiat Oncol Biol Phys 2019;103:11671174.

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    Schmitz JJ, Schmit GD, Atwell TD, et al.. Percutaneous cryoablation of extraabdominal desmoid tumors: a 10-year experience. AJR Am J Roentgenol 2016;207:190195.

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    Ilaslan H, Schils J, Joyce M, et al.. Radiofrequency ablation: another treatment option for local control of desmoid tumors. Skeletal Radiol 2010;39:169173.

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    Cobianchi L, Ravetta V, Viera FT, et al.. The challenge of extraabdominal desmoid tumour management in patients with Gardner’s syndrome: radiofrequency ablation, a promising option. World J Surg Oncol 2014;12:361.

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    Najafi A, Fuchs B, Binkert CA. Mid-term results of MR-guided high-intensity focused ultrasound treatment for relapsing superficial desmoids. Int J Hyperthermia 2019;36:538542.

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    Bucknor MD, Rieke V. MRgFUS for desmoid tumors within the thigh: early clinical experiences. J Ther Ultrasound 2017;5:4.

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    Avedian RS, Bitton R, Gold G, et al.. Is MR-guided high-intensity focused ultrasound a feasible treatment modality for desmoid tumors? Clin Orthop Relat Res 2016;474:697704.

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    Toulmonde M, Pulido M, Ray-Coquard I, et al.. Pazopanib or methotrexate-vinblastine combination chemotherapy in adult patients with progressive desmoid tumours (DESMOPAZ): a non-comparative, randomised, open-label, multicentre, phase 2 study. Lancet Oncol 2019;20:12631272.

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