The standard diagnosis, treatment, and follow-up of gastrointestinal stromal tumors based on guidelines

Gastric Cancer. 2016 Jan;19(1):3-14. doi: 10.1007/s10120-015-0526-8. Epub 2015 Aug 15.

Abstract

Although gastrointestinal stromal tumors (GISTs) are a rare type of cancer, they are the commonest sarcoma in the gastrointestinal tract. Molecularly targeted therapy, such as imatinib therapy, has revolutionized the treatment of advanced GIST and facilitates scientific research on GIST. Nevertheless, surgery remains a mainstay of treatment to obtain a permanent cure for GIST even in the era of targeted therapy. Many GIST guidelines have been published to guide the diagnosis and treatment of the disease. We review current versions of GIST guidelines published by the National Comprehensive Cancer Network, by the European Society for Medical Oncology, and in Japan. All clinical practice guidelines for GIST include recommendations based on evidence as well as on expert consensus. Most of the content is very similar, as represented by the following examples: GIST is a heterogeneous disease that may have mutations in KIT, PDGFRA, HRAS, NRAS, BRAF, NF1, or the succinate dehydrogenase complex, and these subsets of tumors have several distinctive features. Although there are some minor differences among the guidelines--for example, in the dose of imatinib recommended for exon 9-mutated GIST or the efficacy of antigen retrieval via immunohistochemistry--their common objectives regarding diagnosis and treatment are not only to improve the diagnosis of GIST and the prognosis of patients but also to control medical costs. This review describes the current standard diagnosis, treatment, and follow-up of GISTs based on the recommendations of several guidelines and expert consensus.

Keywords: Consensus based; Evidence-based; Gastrointestinal stromal tumor; Guidelines.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antineoplastic Agents / therapeutic use
  • Drug Resistance, Neoplasm
  • Follow-Up Studies
  • Gastrointestinal Neoplasms / diagnosis*
  • Gastrointestinal Neoplasms / epidemiology
  • Gastrointestinal Neoplasms / therapy*
  • Gastrointestinal Stromal Tumors / diagnosis*
  • Gastrointestinal Stromal Tumors / epidemiology
  • Gastrointestinal Stromal Tumors / therapy*
  • Humans
  • Imatinib Mesylate / therapeutic use
  • Mutation
  • Neoplasm Recurrence, Local
  • Phenylurea Compounds / therapeutic use
  • Practice Guidelines as Topic
  • Proto-Oncogene Proteins c-kit / genetics
  • Pyridines / therapeutic use

Substances

  • Antineoplastic Agents
  • Phenylurea Compounds
  • Pyridines
  • regorafenib
  • Imatinib Mesylate
  • Proto-Oncogene Proteins c-kit