Acute Presentations of Gastrointestinal Stromal Tumors

Background: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract (GIT) and are frequently diagnosed incidentally. While most patients with GISTs remain asymptomatic, a subset may present with acute surgical emergencies that require multidisciplinary management. We aimed to conduct a comprehensive narrative review to explore acute GIST presentations and their risk factors, locations, and management. Methods: This review (from inception to July 2026) summarized the spectrum of acute GIST presentations. We explored English publications in PubMed/MEDLINE and Google Scholar. No quantitative analysis or risk of bias assessment was performed for this narrative review. Results: The major symptoms of acute GIST were GI bleeding, intestinal obstruction, intussusception, urinary retention, spontaneous tumor rupture, hemoperitoneum, and perforation. Hemorrhagic shock due to gastrointestinal bleeding or intraperitoneal bleeding, as well as peritonitis due to rupture, is life-threatening. Contrast-enhanced computed tomography (CT) is essential for emergency evaluation, and endoscopic ultrasound with tissue sampling is necessary for diagnostic confirmation. Identifying the genetic inheritance is valuable in limited cases. Emergent surgical resection is the primary treatment for patients with acute GIST presentations; however, the surgical oncologic principles of complete gross resection with an intact pseudocapsule and avoidance of tumor rupture are the same. Tyrosine kinase inhibitors also treat advanced, unresectable, or metastatic disease in selected patients. Conclusions: Recognizing these uncommon but potentially life-threatening GIST presentations facilitate early diagnosis, timely intervention, and improved clinical outcomes.