Challenging differential diagnosis, Unusual setting of medical care, Rare disease
Mehmet Odabasi, Kamil Mehmet Yildiz, Cengiz Eris, Hasan Haci Abuoglu, Emre Gunay, Erkan Ozkan, Ali Aktekin, Bulent Kaya, Tolga Munip Ali Muftuoglu
(Department of Surgery, Haydarpasa Education and Research Hospital, Istanbul, Turkey)
Am J Case Rep 2013; 14:439-443
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