Multiple duodenal stromal tumors revealing type 1 neurofibromatosis: An indication for pancreas-preserving duodenectomy

摘要

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Type 1 neurofibromatosis (von Recklinghausen disease) is known to be associated with GIST.A 56-year-old man with no previous medical history presented with epigastric pain and anemia. The clinical examination showed multiple subcutaneous nodes in the trunk. The upper gastrointestinal endoscopy revealed two submucosal tumors of 20 and 8 mm in the second part of the duodenum, which narrowed the lumen and was associated with 2 additional lesions of less than 1 cm in the third part of the duodenum.