Not All GISTs are Gastric: A Single-center Cohort of Non-gastric GIST

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Objectives: Non-gastric gastrointestinal stromal tumors (GIST) are less common than gastric primaries, but are often considered higher risk.Real-world cohorts focusing on non-gastric GIST with long-term follow-up remain limited. Methods:We conducted a single-center retrospective cohort study of consecutive adults with histologically confirmed GISTs (esophagus, small intestine, and colon/rectum) treated between January 2015 and June 2025.National Institutes of Health (NIH) risk categories documented in pathology reports were recorded, and Armed Forces Institute of Pathology (AFIP) (Miettinen-Lasota) risk groups were calculated when tumor size, mitotic activity (per 5 mm2), and primary site were available.Overall survival (OS) was estimated using the Kaplan-Meier method.Results: Among 114 patients with GIST, 41 (36.0%) had non-gastric primaries (small intestine 73.2%, rectum 19.5%, esophagus 7.3%).Median age was 55 years (range, 30-83); median tumor size was 6.0 cm (range, 0.4-26.0);and median mitotic count was 6/5 mm2 (range, 0-218).At diagnosis, 34 patients had localized disease and 7 had de novo metastatic disease; during follow-up, 10/34 (29.4%) patients developed recurrent metastasis, resulting in 17/41 (41.5%) patients with metastatic disease overall.NIH risk was available for 37/41 (90.2%) patients; of these, 22/37 (59.5%) were high risk.AFIP risk could be calculated in 26/41 (63.4%) patients; of these, 15/26 (57.7%) were classified as high risk.Metastatic sites most commonly involved the liver (47%) and the peritoneum (41%).After a median follow-up of 77 months, 4 of 41 (9.8%) patients had died; the 10-year OS was 86%. Conclusion:In this real-world, non-gastric GIST cohort, high-risk disease was common, and metastases predominantly involved the liver and peritoneum.Favorable long-term survival appears achievable with high-quality surgery and guideline-consistent use of imatinib in routine practice.

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