GIST Treatment Standard-of-Care Guidelines

The following consensus documents summarize expert opinion about treatment for gastrointestinal stromal tumor (GIST cancer).  Patients should become familiar with these guidelines for the standard-of-care treatment of GIST.

In a nutshell, here are the basic points about GIST treatment.

  • Surgery to remove GIST is the first step when possible.  Surgery is potentially curative for primary GISTs that have not metastasized, but the probability of recurrence depends on tumor characteristics.
  • Tumors that are initially inoperable may be pre-treated with imatinib to improve resectability (called neoadjuvant therapy).
  • Following complete removal of a primary GIST, patients with a higher risk of tumor recurrence may consider adjuvant (preventive) treatment with imatinib.
  • Patients with metastatic GIST disease, even if removed, definitely need drug treatment to maintain disease control.
  • For patients whose GIST tumors become resistant to imatinib, sunitinib is a second-line drug treatment.
  • Regorafenib is FDA-approved as a third-line drug for patients whose tumors are not responding to imatinib or sunitinib.
  • Some drugs approved for other conditions may be prescribed off-label for GIST.
  • Additional drugs are under development and/or being tested in clinical trials.

NCCN Patient Guideline for Soft Tissue Sarcoma 2018 – free access online to pdf (downloadable).  Chapter 6 (pages 46-52) covers GIST.

NCCN Task Force Report: Optimal Management of Patients with GIST — 2010 Update.  This is a long (40-page pdf) and detailed guidelines booklet written by a panel of experts.  It contains plenty of useful information to guide oncologists in treatment of GIST.  Free access after a free registration.

NCCN Task Force Report: Optimal Management of Patients with GIST — 2007 Update of the NCCN Clinical Practice Guidelines.  This earlier NCCN guide includes some information such as drug interactions and side effects of special concern to patients that is not found in the 2010 update.  Free access after a free registration.

NCCN Soft Tissue Sarcoma Guidelines   This document, updated at least annually, includes flow charts for GIST diagnosis  and treatment.  Free access after a free registration.

European Society for Medical Oncology GIST guidelines  Annals of Oncology 2012;  23(suppl_7): p. vii49-55vii (See newer version below.)

European Society for Medical Oncology GIST treatment guidelines  2018.    Free access.

The European Sarcoma Network Working Group, Annals of Oncology, Volume 29, Issue Supplement_4, October 2018, “Gastrointestinal stromal tumors: ESMO-EUROCAN Clinical Practice Guidelines for diagnosis, treatment and follow-up”   Pages iv68–iv78, https://doi.org/10.1093/annonc/mdy095 

French National Federation of Cancer Centers consensus guidelines written in French (Recommandations pour la prise en charge des tumeurs stromales gastro-intestinales (GIST)

Korean GIST Guidelines.  “Clinical practice guideline for accurate diagnosis and effective treatment of gastrointestinal stromal tumor in Korea” by  Kang YK, Kim KM, Sohn T, Choi D, Kang HJ, Ryu MH, Kim WH, Yang HK.  J Korean Med Sci. 2010 Nov;25(11):1543-52.  PubMed PMID: 21060741. Free access

Canadian Advisory Committee on GIST statement Gastrointestinal stromal tumours: Consensus statement on diagnosis and treatment (Blackstein et al, 2006).

Clinical practice guidelines for gastrointestinal stromal tumor (GIST) in Japan: English version.
Nishida T and GIST Guideline Subcommittee.
Int J Clin Oncol. 2008 Oct;13(5):416-30. Epub 2008 Oct 23. PubMed PMID: 18946752.

Guidelines for the Management of Gastrointestinal Stromal Tumours (published in Clinical Sarcoma Research 2017)

 

Future GIST Treatment Trends

Because imatinib (Gleevec) and sunitinib (Sutent) do not kill all GIST cancer cells, new treatment ideas under development include:

  • combination trials of imatinib or sunitinib plus other drugs
  • imatinib plus surgery in specified sequences
  • heat shock protein inhibitors
  • other tyrosine kinase inhibitors including sorafenib, nilotinib, dasatinib, etc.
  • inhibitors of downstream pathways such as PI3K, mTOR, MAPK
  • cell cycle  inhibitors
  • proteasome inhibitors