Background: Neuroendocrine tumor of the ampulla of Vater is extremely rare and is generally a low-grade endocrine cell tumor. The merits of radical vs. local resection remain uncertain.
Case Report: A 45-year-old female patient presented with abdominal pain lasting for 2 months. Papilla that was tumor-like macroscopically was seen in the second part of the duodenum in endoscopic retrograde cholangiopancreatography. Biopsy was histologically confirmed as a low-grade neuroendocrine tumor. No lymphadenopathy or visceral metastasis was found on an abdominal CT scan, In-111 octreotide scan, and EUS. The ampulla was removed by endoscopic snare papillectomy. All margins of resection were negative for tumor.
Conclusions: Endoscopic snare papillectomy may be the first step in the management of neuroendocrine tumors of the ampulla of Vater in high-risk surgical candidates and selected patients such as those with a well differentiated, low-grade, small tumor without regional/ distant metastasis. However, it can also be used in younger patients who wish to avoid surgical resection.

Keywords: Endoscopic retrograde cholangiopancreaticography, endoscopic papillectomy, Ampulla of Vater, neuroendocrine tumor