NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022

Author:

von Mehren Margaret1,Kane John M.2,Riedel Richard F.3,Sicklick Jason K.4,Pollack Seth M.5,Agulnik Mark6,Bui Marilyn M.7,Carr-Ascher Janai8,Choy Edwin9,Connelly Mary10,Dry Sarah11,Ganjoo Kristen N.12,Gonzalez Ricardo J.7,Holder Ashley13,Homsi Jade14,Keedy Vicki15,Kelly Ciara M.16,Kim Edward17,Liebner David10,McCarter Martin18,McGarry Sean V.19,Mesko Nathan W.20,Meyer Christian21,Pappo Alberto S.22,Parkes Amanda M.23,Petersen Ivy A.24,Poppe Matthew25,Schuetze Scott26,Shabason Jacob27,Spraker Matthew B.28,Zimel Melissa29,Bergman Mary Anne30,Sundar Hema30,Hang Lisa E.30

Affiliation:

1. 1Fox Chase Cancer Center;

2. 2Roswell Park Comprehensive Cancer Center;

3. 3Duke Cancer Institute;

4. 4UC San Diego Moores Cancer Center;

5. 5Robert H. Lurie Comprehensive Cancer Center of Northwestern University;

6. 6City of Hope National Medical Center;

7. 7Moffitt Cancer Center;

8. 8UC Davis Comprehensive Cancer Center;

9. 9Massachusetts General Hospital Cancer Center;

10. 10The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute;

11. 11UCLA Jonsson Comprehensive Cancer Center;

12. 12Stanford Cancer Institute;

13. 13O’Neal Comprehensive Cancer Center at UAB;

14. 14UT Southwestern Simmons Comprehensive Cancer Center;

15. 15Vanderbilt-Ingram Cancer Center;

16. 16Memorial Sloan Kettering Cancer Center;

17. 17Fred Hutchinson Cancer Center;

18. 18University of Colorado Cancer Center;

19. 19Fred & Pamela Buffett Cancer Center;

20. 20Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute;

21. 21The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins;

22. 22St. Jude Children’s Research Hospital/University of Tennessee Health Science Center;

23. 23University of Wisconsin Carbone Cancer Center;

24. 24Mayo Clinic Cancer Center;

25. 25Huntsman Cancer Institute at the University of Utah;

26. 26University of Michigan Rogel Cancer Center;

27. 27Abramson Cancer Center at the University of Pennsylvania;

28. 28Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine;

29. 29UCSF Helen Diller Family Comprehensive Cancer Center; and

30. 30National Comprehensive Cancer Network.

Abstract

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.

Publisher

Harborside Press, LLC

Subject

Oncology

Reference146 articles.

1. Evaluation of nilotinib in advanced GIST previously treated with imatinib and sunitinib;Cauchi;Cancer Chemother Pharmacol,2012

2. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors;Hirota;Science,1998

3. Duodenal-jejunal flexure GI stromal tumor frequently heralds somatic NF1 and Notch pathway mutations;Burgoyne;JCO Precis Oncol,2017

4. Clinical management of gastrointestinal stromal tumors: before and after STI-571;Dematteo;Hum Pathol,2002

5. Avapritinib in advanced PDGFRA D842V-mutant gastrointestinal stromal tumour (NAVIGATOR): a multicentre, open-label, phase 1 trial;Heinrich;Lancet Oncol,2020

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