Simultaneous occurrence of an ampullary neuroendocrine tumor and multiple duodenal/jejunal gastrointestinal stromal tumors in a patient with neurofibromatosis type 1
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- KITAJIMA Ryo
- Second Department of Surgery, Hamamatsu University School of Medicine
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- MORITA Yoshifumi
- Second Department of Surgery, Hamamatsu University School of Medicine
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- FURUHASHI Satoru
- Second Department of Surgery, Hamamatsu University School of Medicine
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- KIUCHI Ryota
- Second Department of Surgery, Hamamatsu University School of Medicine
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- TAKEDA Makoto
- Second Department of Surgery, Hamamatsu University School of Medicine
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- KIKUCHI Hirotoshi
- Second Department of Surgery, Hamamatsu University School of Medicine
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- WATANABE Fumitoshi
- Department of Gastroenterology, Hamamatsu South Hospital
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- SUGIMOTO Ken
- First Department of Medicine, Gastroenterology, Hamamatsu University School of Medicine
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- SAKAGUCHI Takanori
- Second Department of Surgery, Hamamatsu University School of Medicine
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- TAKEUCHI Hiroya
- Second Department of Surgery, Hamamatsu University School of Medicine
Bibliographic Information
- Other Title
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- 乳頭部神経内分泌腫瘍と多発小腸GISTを併発した神経線維腫症1型の1例
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Abstract
<p>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.</p>
Journal
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- Nippon Shokakibyo Gakkai Zasshi
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Nippon Shokakibyo Gakkai Zasshi 116 (7), 583-591, 2019-07-10
The Japanese Society of Gastroenterology