Introduction: Gastrointestinal stromal tumors (GISTs) arising in the duodenum represent a rare entity and can be very demanding to manage. The diagnosis can be difficult (as these tumors can be misdiagnosed as pancreatic head tumors), and to treat owing to the complex anatomy of the duodenum and of the pancreatic head. Complete resection may require extensive procedures such as pancreaticodudodenectomy.
Methods and results: An extensive literature review regarding clinico-pathological features, diagnostic investigations and surgical options in the management of duodenal GISTs has been performed.
Conclusions: Duodenal GISTs have uncertain malignant potential, and they may be asymptomatic, or present with abdominal pain or bleeding. A preoperative diagnosis can be difficult to obtain. Endoscopic ultrasound and fine needle aspiration cytology (FNAC) may be helpful. Surgical R0 resection remains the only curative approach. However, owing to the complex anatomy of the duodenum, limited resection is not always feasible. In these cases, extensive procedures such as pancreaticoduodenectomy or pancreas-preserving duodenectomy are the treatment of choice.
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