Gastrointestinal Stromal Tumor: History, Molecular Subtypes, and Risk Stratification
According to the Surveillance, Epidemiology, and End Results Program database, GIST accounts for approximately 2.2% of all malignant gastric tumors.
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pathologists owing to the adoption of digital pathology and discrepancies between updated international guidelines and outdated reimbursement requirements.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.
The claim quantifies the relative frequency of GIST within malignant gastric tumors, underscoring the importance of recognizing and mandating precision diagnostics and optimal therapeutic strategies for this uncommon but significant neoplasm.
Evidence level: Klinikai vizsgálat. Formális klinikai vizsgálati eredmény.
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