Introduction: Familial adenomatous polyposis (FAP) is characterized by the development of duodenal polyposis (DP), which later develops into colonic adenomatous polyps and, eventually, colorectal cancer. Neuroendocrine tumors (NET) are rare in FAP and reports of pancreas-preserving total duodenectomy (PPTD) to treat NET are limited.
Case: A 62-year-old. woman was previously diagnosed with FAP and she underwent a total colectomy and ileorectal anastomosis. Surveillance by upper gastrointestinal endoscopy revealed duodenal polyposis and a 35 mm flat, elevated tumor near the ampulla of Vater. She was diagnosed as having Spigelman stage Ⅳ DP and she underwent PPTD. Histopathology revealed a 7 mm NET G1 in the ampulla of Vater and multiple adenomas.
Discussion: Little is known about duodenal NET G1in FAP patients who undergo PPTD. Close follow-up is necessary.
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