Abstract
A 42-year-old man with neurofibromatosis type 1 (NF1) who visited a hospital because of tarry stool in March 2010 was referred to our hospital because a duodenal tumor was detected by upper gastrointestinal endoscopy. Abdominal CT scan revealed a 50 × 50 × 25 mm tumor from the second to the third part of duodenum. Upper gastrointestinal endoscopy and EUS showed a 21 × 16 mm submucosal tumor with a central depression in the vicinity of the papilla of Vater at the second part of duodenum, and it was proved to be neuroendocrine tumor (NET) by a biopsy. Another submucosal tumor 51 × 31 mm in diameter was present in the third part of duodenum. Pancreaticoduodenectomy was performed in May. The histopathology resulted in NET and gastrointestinal stromal tumor (GIST). The postoperative course was uneventful. The patient was discharged from our hospital on the 19th postoperative day and has been free from recurrence as of 2 years and 8 months after the operation.
Recently clinical cases of NF1 associated with either NET or GIST have sometimes been reported, however, there are few cases of NF1 associated with the both diseases concomitantly. We thus report this case of NF1 with NET and GIST of the duodenum, together with some bibliographic comments.
