Multimedialna platforma wiedzy medycznej Internetowa Księgarnia Medyczna Kalendarium Konferencji
Nowotwory. Journal of Oncology
MENU
Shortcuts Submit an article Author Guidelines Ahead Of Print Editorial Office Manuscript Submission Reviewers 2023 Best Original Paper Award

open access

Vol 70, No 6 (2020)
Other materials agreed with the Editors
Published online: 2020-12-04
View PDF Download PDF file
Get Citation

Advances in the management of gastrointestinal stromal tumors (GISTs)

Monika Dudzisz-Śledź1, Piotr Rutkowski1
DOI: 10.5603/NJO.2020.0055
·
Nowotwory. Journal of Oncology 2020;70(6):280-287.
Affiliations
  1. Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland

open access

Vol 70, No 6 (2020)
Conference addendum
Published online: 2020-12-04

Abstract

Gastrointestinal stromal tumors are rare neoplasms developing from cells of Cajal in the gastrointestinal tract. The mainstay of such tumors treatment is surgery, whenever possible. The therapeutic management of inoperable and metastatic disease is based on tyrosine kinase inhibitors and imatinib is the main drug recommended for first line treatment. The introduction of imatinib and other inhibitors improved survival outcomes for this disease, but due to primary and secondary resistance there is still the urgent need for new medications. This paper presents the progress in the systemic therapy of GISTs based on the latest scientific data. The newly developed agents (ripretinib, avapritinib) meet the need to treat patients after the failure of previously available therapies and those with PDGFRA mutation D842V associated with resistance to imatinib.

Abstract

Gastrointestinal stromal tumors are rare neoplasms developing from cells of Cajal in the gastrointestinal tract. The mainstay of such tumors treatment is surgery, whenever possible. The therapeutic management of inoperable and metastatic disease is based on tyrosine kinase inhibitors and imatinib is the main drug recommended for first line treatment. The introduction of imatinib and other inhibitors improved survival outcomes for this disease, but due to primary and secondary resistance there is still the urgent need for new medications. This paper presents the progress in the systemic therapy of GISTs based on the latest scientific data. The newly developed agents (ripretinib, avapritinib) meet the need to treat patients after the failure of previously available therapies and those with PDGFRA mutation D842V associated with resistance to imatinib.

Full Text:

View PDF Download PDF file
Get Citation

Keywords

gastrointestinal stromal tumor; GIST; tyrosine kinase inhibitor; imatinib; regorafenib; sunitinib; sorafenib; avapritinib; repritinib; BLU-285; DCC-2618

About this article
Title

Advances in the management of gastrointestinal stromal tumors (GISTs)

Journal

Nowotwory. Journal of Oncology

Issue

Vol 70, No 6 (2020)

Article type

Other materials agreed with the Editors

Pages

280-287

Published online

2020-12-04

Page views

643

Article views/downloads

633

DOI

10.5603/NJO.2020.0055

Bibliographic record

Nowotwory. Journal of Oncology 2020;70(6):280-287.

Keywords

gastrointestinal stromal tumor
GIST
tyrosine kinase inhibitor
imatinib
regorafenib
sunitinib
sorafenib
avapritinib
repritinib
BLU-285
DCC-2618

Authors

Monika Dudzisz-Śledź
Piotr Rutkowski

References (39)
  1. Søreide K, Sandvik OM, Søreide JA, et al. Global epidemiology of gastrointestinal stromal tumours (GIST): A systematic review of population-based cohort studies. Cancer Epidemiol. 2016; 40: 39–46.
    CrossRefPubMed
  2. Miettinen M, Lasota J. Gastrointestinal stromal tumors--definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001; 438(1): 1–12.
    CrossRefPubMed
  3. Ahmed M. Recent advances in the management of gastrointestinal stromal tumor. World J Clin Cases. 2020; 8(15): 3142–3155.
    CrossRefPubMed
  4. Rutkowski P, Szumera-Ciećkiewicz A. Gastrointestinal stromal tumours (GIST) — 2018. Oncol Clin Pract. 2019; 14(6): 399–407.
    CrossRef
  5. Graadt van Roggen JF, van Velthuysen ML, Hogendoorn PC. The histopathological differential diagnosis of gastrointestinal stromal tumours. J Clin Pathol. 2001; 54(2): 96–102.
    CrossRefPubMed
  6. Casali PG, Abecassis N, Aro HT, et al. ESMO Guidelines Committee and EURACAN, ESMO Guidelines Committee and EURACAN. Gastrointestinal stromal tumours: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018; 29(Suppl 4): iv68–iv78.
    CrossRefPubMed
  7. Rutkowski P, Hompes D. Combined Therapy of Gastrointestinal Stromal Tumors. Surg Oncol Clin N Am. 2016; 25(4): 735–759.
    CrossRefPubMed
  8. Joensuu H, Eriksson M, Sundby Hall K, et al. One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA. 2012; 307(12): 1265–1272.
    CrossRefPubMed
  9. Joensuu H, Eriksson M, Hall KS, et al. Three versus one year of adjuvant imatinib for high-risk gastrointestinal stromal tumor (GIST): Survival analysis of a randomized trial after 10 years of follow-up. Journal of Clinical Oncology. 2020; 38(15_suppl): 11503–11503.
    CrossRef
  10. Rutkowski P, Ziętek M, Cybulska-Stopa B, et al. The analysis of 3-year adjuvant therapy with imatinib in patients with high-risk molecular profiled gastrointestinal stromal tumors (GIST) treated in routine practice. Eur J Surg Oncol. 2020 [Epub ahead of print].
    CrossRefPubMed
  11. Wang D, Zhang Q, Blanke CD, et al. Phase II trial of neoadjuvant/adjuvant imatinib mesylate for advanced primary and metastatic/recurrent operable gastrointestinal stromal tumors: long-term follow-up results of Radiation Therapy Oncology Group 0132. Ann Surg Oncol. 2012; 19(4): 1074–1080.
    CrossRefPubMed
  12. Rutkowski P, Gronchi A, Hohenberger P, et al. Neoadjuvant imatinib in locally advanced gastrointestinal stromal tumors (GIST): the EORTC STBSG experience. Ann Surg Oncol. 2013; 20(9): 2937–2943.
    CrossRefPubMed
  13. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002; 347(7): 472–480.
    CrossRefPubMed
  14. Blanke CD, Demetri GD, von Mehren M, et al. Long-term results from a randomized phase II trial of standard- versus higher-dose imatinib mesylate for patients with unresectable or metastatic gastrointestinal stromal tumors expressing KIT. J Clin Oncol. 2008; 26(4): 620–625.
    CrossRefPubMed
  15. Rutkowski P, Andrzejuk J, Bylina E, et al. What are the current outcomes of advanced gastrointestinal stromal tumors: who are the long-term survivors treated initially with imatinib? Med Oncol. 2013; 30(4): 765.
    CrossRefPubMed
  16. Brzozowska M, Wierzba W, Szafraniec-Buryło S, et al. Real-World Evidence of Patient Outcome Following Treatment of Advanced Gastrointestinal Stromal Tumor (GIST) with Imatinib, Sunitinib, and Sorafenib in Publicly Funded Health Care in Poland. Med Sci Monit. 2019; 25: 3846–3853.
    CrossRefPubMed
  17. Demetri GD, van Oosterom AT, Garrett CR, 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(9544): 1329–1338.
    CrossRefPubMed
  18. Reichardt P, Kang YK, Rutkowski P, et al. Clinical outcomes of patients with advanced gastrointestinal stromal tumors: safety and efficacy in a worldwide treatment-use trial of sunitinib. Cancer. 2015; 121(9): 1405–1413.
    CrossRefPubMed
  19. Sutent (sunitinib) SmPC. https://www.ema.europa.eu/en/documents/product-information/sutent-epar-product-information_en.pdf.
  20. Montemurro M, Gelderblom H, Bitz U, et al. Sorafenib as third- or fourth-line treatment of advanced gastrointestinal stromal tumour and pretreatment including both imatinib and sunitinib, and nilotinib: A retrospective analysis. Eur J Cancer. 2013; 49(5): 1027–1031.
    CrossRefPubMed
  21. Rutkowski P, Jagielska B, Andrzejuk J, et al. The analysis of the long-term outcomes of sorafenib therapy in routine practice in imatinib and sunitinib resistant gastrointestinal stromal tumors (GIST). Contemp Oncol (Pozn). 2017; 21(4): 285–289.
    CrossRefPubMed
  22. Demetri G, Reichardt P, Kang YK, 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(9863): 295–302.
    CrossRef
  23. Kang YK, Ryu MH, Yoo C, et al. Resumption of imatinib to control metastatic or unresectable gastrointestinal stromal tumours after failure of imatinib and sunitinib (RIGHT): a randomised, placebo-controlled, phase 3 trial. Lancet Oncol. 2013; 14(12): 1175–1182.
    CrossRefPubMed
  24. NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines), Gastrointestinal Stromal Tumors (GISTs), version 1.2021. 10.2020. 2021.
  25. https://www.clinicaltrials.gov/ct2/results?cond=GIST&term=&cntry=&state=&city=&dist=.
  26. Bauer S, Rutkowski P, Hohenberger P, et al. Long-term follow-up of patients with GIST undergoing metastasectomy in the era of imatinib -- analysis of prognostic factors (EORTC-STBSG collaborative study). Eur J Surg Oncol. 2014; 40(4): 412–419.
    CrossRefPubMed
  27. Du CY, Zhou Ye, Song C, et al. Is there a role of surgery in patients with recurrent or metastatic gastrointestinal stromal tumours responding to imatinib: a prospective randomised trial in China. Eur J Cancer. 2014; 50(10): 1772–1778.
    CrossRefPubMed
  28. Tielen R, Verhoef C, van Coevorden F, et al. Surgery after treatment with imatinib and/or sunitinib in patients with metastasized gastrointestinal stromal tumors: is it worthwhile? World J Surg Oncol. 2012; 10(2): 111–155.
    CrossRefPubMed
  29. AYVAKYT (avapritinib) SMPC. https://www.ema.europa.eu/en/documents/product-information/ayvakyt-epar-product-information_en.pdf.
  30. AYVAKYT (avapritinib) prescribing information. https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/212608s000lbl.pdf.
  31. Evans EK, Gardino AK, Kim JL, et al. A precision therapy against cancers driven by mutations. Sci Transl Med. 2017; 9(414).
    CrossRefPubMed
  32. Heinrich MC, Jones RL, von Mehren M, et al. Avapritinib in advanced PDGFRA D842V-mutant gastrointestinal stromal tumour (NAVIGATOR): a multicentre, open-label, phase 1 trial. Lancet Oncol. 2020; 21(7): 935–946.
    CrossRefPubMed
  33. Jones RL, Serrano C, von Me, et al. Long-term efficacy, tolerability and overall survival in patients with unresectable or metastatic PDGFRA D842V-mutant gastrointestinal stromal tumor treated with avapritinib: NAVIGATOR phase 1 trial update. Ann Oncol. 2020; 31(Suppl 4): abstract 1621MO.
  34. https://clinicaltrials.gov/ct2/show/NCT03465722.
  35. https://www.prnewswire.com/news-releases/blueprint-medicines-announces-top-line-results-from-phase-3-voyager-trial-of-avapritinib-versus-regorafenib-in-patients-with-advanced-gastrointestinal-stromal-tumor-301048058.html.
  36. QINLOCK (ripretinib) prescribing information. https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213973s000lbl.pdf.
  37. Smith BD, Kaufman MD, Lu WP, et al. Ripretinib (DCC-2618) Is a Switch Control Kinase Inhibitor of a Broad Spectrum of Oncogenic and Drug-Resistant KIT and PDGFRA Variants. Cancer Cell. 2019; 35(5): 738–751.e9.
    CrossRefPubMed
  38. Blay JY, Serrano C, Heinrich M, et al. Ripretinib in patients with advanced gastrointestinal stromal tumours (INVICTUS): a double-blind, randomised, placebo-controlled, phase 3 trial. Lancet Oncol. 2020; 21(7): 923–934.
    CrossRef
  39. George S, Heinrich M, Chi P, et al. Initial results of phase I study of DCC-2618, a broad-spectrum KIT and PDGFRa inhibitor, in patients (pts) with gastrointestinal stromal tumor (GIST) by number of prior regimens. Annals of Oncology. 2018; 29: viii576–viii577.
    CrossRef

Regulations

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

Wydawcą serwisu jest VM Media Group sp. z o.o., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl