Navigating the complex landscape of duodenal gastrointestinal stromal tumor: Innovations in diagnosis and treatment.

Duodenal gastrointestinal stromal tumors (dGISTs) are a rare subset of GISTs characterized by distinct anatomical constraints and clinical management challenges. Although sharing common molecular drivers with GISTs at other sites, dGISTs differ substantially in surgical complexity, recurrence risk, and treatment decision-making due to their proximity to the pancreas, biliary tract, and major vascular structures. Advances in imaging, endoscopic evaluation, and molecular profiling have improved diagnostic accuracy; however, most evidence remains extrapolated from non-duodenal cohorts. Surgical resection with negative margins remains the cornerstone of curative treatment, with increasing emphasis on procedure selection, particularly the balance between limited resection and pancreaticoduodenectomy. Neoadjuvant imatinib has been increasingly adopted for locally advanced or anatomically complex tumors to facilitate resectability and organ preservation, although optimal treatment duration and patient selection remain controversial. Systemic therapy is guided primarily by KIT and PDGFRA mutation status, while emerging agents and ongoing clinical trials aim to address resistance and high-risk disease. Nevertheless, duodenum-specific data remain limited, and consensus on optimal management strategies is lacking. This review summarizes current evidence and evolving strategies in the diagnosis and treatment of dGISTs, with a focus on surgical decision-making, perioperative systemic therapy, and ongoing clinical trials, and highlights critical unmet needs requiring future investigation.