Journal of Gastric Cancer

The Korean Gastric Cancer Association (대한위암학회)
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Gastrointestinal Stromal Tumor: History, Molecular Subtypes, and Risk Stratification

  • In Hye Song (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Soomin Ahn (Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Hyung-Don Kim (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jeong-Hyeon Jo (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jinho Shin (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Min-Hee Ryu (Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Young Soo Park (Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine)
  • Received : 2025.11.14
  • Accepted : 2025.12.11
  • Published : 2026.04.01
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Abstract

The gastrointestinal stromal tumor (GIST) is one of the most common mesenchymal tumors of the gastrointestinal tract. Between the 1990s and early 2000s, GIST was identified as a tumor characterized by KIT or PDGFRA mutations, resulting in imatinib being established as an effective targeted therapy. However, with advances in molecular diagnostics, approximately 10%-15% of GISTs have been reported to harbor alternative mutations, such as those in the succinate dehydrogenase subunit genes and BRAF, leading to the development of additional targeted therapies. GISTs exhibit a wide spectrum of clinical behaviors, ranging from indolent to highly aggressive, prompting the development of diverse risk classification systems. However, multiple systems remain in use, leading to inconsistent pathologic reports. Moreover, the mitotic counting method-a key factor in risk stratification-has become a major source of confusion among pathologists owing to the adoption of digital pathology and discrepancies between updated international guidelines and outdated reimbursement requirements. These inconsistencies have hindered pathologic reporting and communication between pathologists and clinicians. This review comprehensively overviews the historical background, molecular subtypes, and risk classification systems of GIST, focusing on evolving issues in mitotic rate evaluation and the application of risk classification systems in clinical practice.

Keywords

Acknowledgement

We would like to thank Editage (www.editage.co.kr) for English language editing.

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