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Gastrointestinal stromal tumor: a review of current and emerging therapies

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Abstract

Gastrointestinal stromal tumors (GIST) are rare neoplasms arising from the interstitial cell of Cajal in the gastrointestinal tract. Two thirds of GIST in adult patients have c-Kit mutation and smaller fractions have platelet derived growth factor receptor alpha (PDGFRA) mutation. Surgery is the only curative treatment for localized disease. Imatinib improves survival when used adjuvantly and in advanced disease. Several targeted therapies have also improved survival in GIST patients after progression on imatinib including sunitinib and regorafenib. Recently, United States Federal and Drug Administration (FDA) approved two new tyrosine kinase inhibitors for the treatment of heavily pretreated advanced/unresectable GIST including avapritinib (a selective inhibitor for PDGFRA exon 18 mutation including D842V mutations) and ripretinib (a broad-spectrum kinase inhibitor of c-Kit and PDGFRA). In this article, we will provide a comprehensive review of GIST including the current standard of care treatment and exploring future paradigm shifts in therapy.

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  1. Department of Oncology, Karmanos Cancer Institute, School of Medicine, Wayne State University, Detroit, MI, USA

    Bayan Al-Share, Abdulrahman Alloghbi, Mohammed Najeeb Al Hallak, Hafiz Uddin, Asfar Azmi, Ramzi M. Mohammad, Steve H Kim, Anthony F. Shields & Philip A Philip

  2. Department of Pharmacology, School of Medicine, Wayne State University, Detroit, MI, USA

    Philip A Philip

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  1. Bayan Al-Share
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Asfar Azmi—funding from Janssen and Karyopharm Therapeutics Inc. Consultant for Guidepoint and GLG. Speaker bureau at Karyopharm Therapeutics Inc. All other authors declare that they have no conflict of interest.

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Al-Share, B., Alloghbi, A., Al Hallak, M.N. et al. Gastrointestinal stromal tumor: a review of current and emerging therapies. Cancer Metastasis Rev 40, 625–641 (2021). https://doi.org/10.1007/s10555-021-09961-7

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