Neuroendocrine tumors (NETs) that arise from neuroendocrine cells can develop in most organs; however, it is rarely found in the duodenal papilla. Conversely, gastrointestinal stromal tumors (GISTs), which are mostly asymptomatic and detected incidentally, are usually found in the stomach and very rarely occur metachronously with NETs. A 42-year-old female with no specific underlying disease underwent gastroscopy due to epigastric pain. Biopsy of enlarged major and minor duodenal papilla confirmed the diagnosis of a NET. Endoscopic papillectomy of the major and minor papillae was performed. Multiple duodenal and jejunal submucosal nodules were seen on biliary CT performed at the 30 months follow-up. Pylorus-preserving pancreaticoduodenectomy was performed due to the suspicion of multiple recurrent NETs and muscularis propria involvement on endoscopic ultrasound. Surgical specimen biopsy confirmed the diagnosis of multiple duodenal and jejunal GIST lesions and a metastatic NET in the duodenal lymph node. We report a rare case of a GIST detected in the duodenum during follow-up after the diagnosis and papillectomy of duodenal papilla NET.
Keywords: Gastrointestinal stromal tumors; Neuroendocrine tumors.