[Simultaneous occurrence of an ampullary neuroendocrine tumor and multiple duodenal/jejunal gastrointestinal stromal tumors in a patient with neurofibromatosis type 1].

A 60-year-old female visited our hospital because of the identification of two duodenal tumors on upper gastrointestinal endoscopy performed for the investigation of anemia. The oral ampullary tumor was proven to be a neuroendocrine tumor (NET) on endoscopic biopsy. However, biopsy was not performed for the anal submucosal tumor (SMT) in the third duodenal portion because the tumor was included in the planned resection area. Multiple dermal soft tumors and café-au-lait spots were noted;her mother and daughter showed the same symptoms. The patient was diagnosed with neurofibromatosis type 1 (NF1). Laparotomy revealed more than 10 nodules at the serosal surface of the duodenum and proximal jejunum. Pancreaticoduodenectomy was performed with an additional 30-cm length of the jejunum that included most of the protruding tumors. Pathologically, the ampullary tumor and the other duodenal/jejunal SMTs were NET G2 and gastrointestinal stromal tumors (GISTs), respectively. Here, we report the rare case of simultaneous occurrence of an ampullary NET and multiple duodenal/jejunal GISTs in a patient with NF1.