Soreide K, Sandvik OM, Soreide JA, Giljaca V, Jureckova A, Bulusu VR. Global epidemiology of gastrointestinal stromal tumours (GIST): A systematic review of population-based cohort studies. Cancer Epidemiol. 2016;40:39–46.

Article  PubMed  Google Scholar 

Nishida T, Yoshinaga S, Takahashi T, Naito Y. Recent Progress and Challenges in the Diagnosis and Treatment of Gastrointestinal Stromal Tumors. Cancers. 2021;13:3158.

Patel SR, Reichardt P. An updated review of the treatment landscape for advanced gastrointestinal stromal tumors. Cancer. 2021;127:2187–95.

Article  PubMed  Google Scholar 

Antonescu CR, Sommer G, Sarran L, Tschernyavsky SJ, Riedel E, Woodruff JM, et al. Association of KIT exon 9 mutations with nongastric primary site and aggressive behavior: KIT mutation analysis and clinical correlates of 120 gastrointestinal stromal tumors. Clinical cancer research : an official journal of the American Association for. Cancer Res. 2003;9:3329–37.

CAS  Google Scholar 

Kang HJ, Nam SW, Kim H, Rhee H, Kim NG, Kim H, et al. Correlation of KIT and platelet-derived growth factor receptor alpha mutations with gene activation and expression profiles in gastrointestinal stromal tumors. Oncogene. 2005;24:1066–74.

Article  CAS  PubMed  Google Scholar 

Steigen SE, Eide TJ, Wasag B, Lasota J, Miettinen M. Mutations in gastrointestinal stromal tumors-a population-based study from Northern Norway. APMIS. 2007;115:289–98.

Article  CAS  PubMed  Google Scholar 

Emile JF, Brahimi S, Coindre JM, Bringuier PP, Monges G, Samb P, et al. Frequencies of KIT and PDGFRA mutations in the MolecGIST prospective population-based study differ from those of advanced GISTs. Med Oncol. 2012;29:1765–72.

Article  CAS  PubMed  Google Scholar 

Joensuu H, Roberts PJ, Sarlomo-Rikala M, Andersson LC, Tervahartiala P, Tuveson D, et al. Effect of the tyrosine kinase inhibitor STI571 in a patient with a metastatic gastrointestinal stromal tumor. N Engl J Med. 2001;344:1052–6.

Article  CAS  PubMed  Google Scholar 

Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006;368:1329–38.

Article  CAS  PubMed  Google Scholar 

Demetri GD, Reichardt P, Kang YK, Blay JY, Rutkowski P, Gelderblom H, et al. Efficacy and safety of regorafenib for advanced gastrointestinal stromal tumours after failure of imatinib and sunitinib (GRID): an international, multicentre, randomised, placebo-controlled, phase 3 trial. Lancet. 2013;381:295–302.

Article  CAS  PubMed  Google Scholar 

Blay JY, Serrano C, Heinrich MC, Zalcberg J, Bauer S, Gelderblom H, et al. Ripretinib in patients with advanced gastrointestinal stromal tumours (INVICTUS): a double-blind, randomised, placebo-controlled, phase 3 trial. Lancet Oncol. 2020;21:923–34.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Chen LL, Trent JC, Wu EF, Fuller GN, Ramdas L, Zhang W, et al. A missense mutation in KIT kinase domain 1 correlates with imatinib resistance in gastrointestinal stromal tumors. Cancer Res. 2004;64:5913–9.

Article  CAS  PubMed  Google Scholar 

Heinrich MC, Corless CL, Blanke CD, Demetri GD, Joensuu H, Roberts PJ, et al. Molecular correlates of imatinib resistance in gastrointestinal stromal tumors. J Clin Oncol. 2006;24:4764–74.

Article  CAS  PubMed  Google Scholar 

Antonescu CR, Besmer P, Guo T, Arkun K, Hom G, Koryotowski B, et al. Acquired resistance to imatinib in gastrointestinal stromal tumor occurs through secondary gene mutation. Clin Cancer Res. 2005;11:4182–90.

Article  CAS  PubMed  Google Scholar 

Debiec-Rychter M, Cools J, Dumez H, Sciot R, Stul M, Mentens N, et al. Mechanisms of resistance to imatinib mesylate in gastrointestinal stromal tumors and activity of the PKC412 inhibitor against imatinib-resistant mutants. Gastroenterology. 2005;128:270–9.

Article  CAS  PubMed  Google Scholar 

Du J, Wang S, Wang R, Wang SY, Han Q, Xu HT, et al. Identifying Secondary Mutations in Chinese Patients with Imatinib-Resistant Gastrointestinal Stromal Tumors (GISTs) by Next Generation Sequencing (NGS). Pathol Oncol Res. 2020;26:91–100.

Article  CAS  PubMed  Google Scholar 

Cao J, Wei J, Yang P, Zhang T, Chen Z, He F, et al. Genome-scale CRISPR-Cas9 knockout screening in gastrointestinal stromal tumor with Imatinib resistance. Mol Cancer. 2018;17:121.

Article  PubMed  PubMed Central  Google Scholar 

Huang WK, Akcakaya P, Gangaev A, Lee L, Zeljic K, Hajeri P, et al. miR-125a-5p regulation increases phosphorylation of FAK that contributes to imatinib resistance in gastrointestinal stromal tumors. Exp Cell Res. 2018;371:287–96.

Article  CAS  PubMed  Google Scholar 

Tsai M, Valent P, Galli SJ. KIT as a master regulator of the mast cell lineage. J allergy Clin Immunol. 2022;149:1845–54.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Lennartsson J, Ronnstrand L. Stem cell factor receptor/c-Kit: from basic science to clinical implications. Physiological Rev. 2012;92:1619–49.

Article  CAS  Google Scholar 

Foster BM, Zaidi D, Young TR, Mobley ME, Kerr BA. CD117/c-kit in Cancer Stem Cell-Mediated Progression and Therapeutic Resistance. Biomedicines. 2018;6:31.

Nakahara M, Isozaki K, Hirota S, Miyagawa J, Hase-Sawada N, Taniguchi M, et al. A novel gain-of-function mutation of c-kit gene in gastrointestinal stromal tumors. Gastroenterology. 1998;115:1090–5.

Article  CAS  PubMed  Google Scholar 

Furitsu T, Tsujimura T, Tono T, Ikeda H, Kitayama H, Koshimizu U, et al. Identification of mutations in the coding sequence of the proto-oncogene c-kit in a human mast cell leukemia cell line causing ligand-independent activation of c-kit product. J Clin Investig. 1993;92:1736–44.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science. 1998;279:577–80.

Article  CAS  PubMed  Google Scholar 

Bosbach B, Rossi F, Yozgat Y, Loo J, Zhang JQ, Berrozpe G, et al. Direct engagement of the PI3K pathway by mutant KIT dominates oncogenic signaling in gastrointestinal stromal tumor. Proc Natl Acad Sci USA. 2017;114:E8448–E57.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Henry JR, Kaufman MD, Peng SB, Ahn YM, Caldwell TM, Vogeti L, et al. Discovery of 1-(3,3-dimethylbutyl)-3-(2-fluoro-4-methyl-5-(7-methyl-2-(methylamino)pyrido[2,3-d]pyrimidin-6-yl)phenyl)urea (LY3009120) as a pan-RAF inhibitor with minimal paradoxical activation and activity against BRAF or RAS mutant tumor cells. J Med Chem. 2015;58:4165–79.

Article  CAS  PubMed  Google Scholar 

Hall-Jackson CA, Eyers PA, Cohen P, Goedert M, Boyle FT, Hewitt N, et al. Paradoxical activation of Raf by a novel Raf inhibitor. Chem Biol. 1999;6:559–68.

Article  CAS  PubMed  Google Scholar 

Peng SB, Henry JR, Kaufman MD, Lu WP, Smith BD, Vogeti S, et al. Inhibition of RAF Isoforms and Active Dimers by LY3009120 Leads to Anti-tumor Activities in RAS or BRAF Mutant Cancers. Cancer Cell. 2015;28:384–98.

Article  CAS  PubMed  Google Scholar 

Zhu G, Shi J, Zhang S, Guo Y, Huang L, Zhao H, et al. Loss of PI3 kinase association improves the sensitivity of secondary mutation of KIT to Imatinib. Cell Biosci. 2020;10:16.

Article  PubMed  PubMed Central  Google Scholar 

Theou-Anton N, Tabone S, Brouty-Boye D, Saffroy R, Ronnstrand L, Lemoine A, et al. Co expression of SCF and KIT in gastrointestinal stromal tumours (GISTs) suggests an autocrine/paracrine mechanism. Br J Cancer. 2006;94:1180–5.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Blume-Jensen P, Wernstedt C, Heldin CH, Ronnstrand L. Identification of the major phosphorylation sites for protein kinase C in kit/stem cell factor receptor in vitro and in intact cells. J Biol Chem. 1995;270:14192–200.

Article  CAS  PubMed  Google Scholar 

Blay JY, Kang YK, Nishida T, von Mehren M. Gastrointestinal stromal tumours. Nat Rev Dis Prim. 2021;7:22.

Article  PubMed  Google Scholar 

Mol CD, Dougan DR, Schneider TR, Skene RJ, Kraus ML, Scheibe DN, et al. Structural basis for the autoinhibition and STI-571 inhibition of c-Kit tyrosine kinase. J Biol Chem. 2004;279:31655–63.

Article  CAS  PubMed  Google Scholar 

Corless CL, Heinrich MC. Molecular pathobiology of gastrointestinal stromal sarcomas. Annu Rev Pathol. 2008;3:557–86.

Article  CAS  PubMed  Google Scholar 

Voytyuk O, Lennartsson J, Mogi A, Caruana G, Courtneidge S, Ashman LK, et al. Src family kinases are involved in the differential signaling from two splice forms of c-Kit. J Biol Chem. 2003;278:9159–66.

Article  CAS  PubMed  Google Scholar 

Sun J, Pedersen M, Ronnstrand L. The D816V mutation of c-Kit circumvents a requirement for Src family kinases in c-Kit signal transduction. J Biol Chem. 2009;284:11039–47.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Lindblad O, Kazi JU, Ronnstrand L, Sun J. PI3 kinase is indispensable for oncogenic transformation by the V560D mutant of c-Ki