Neuroendocrine carcinoma of the ampulla of Vater: a case report, review and recommendations

Neuroendocrine tumor (NET) of the ampulla of Vater is a rare presentation of biliary obstruction. Here, we present a case of NET and discuss the current recommendations that necessitate different management and surgical treatment than other sites. A 56-year-old Caucasian female presented with 2 years of right upper quadrant pain. Workup revealed a well-differentiated 2.2 cm NET at the ampulla with 0 MF/10 HPF, Stage 1A T2NOMO. Whipple procedure performed with 2.2 cm ampullary NET with 1 of the 15 lymph node metastases and <2 mitoses in 10 HPF. TNM classification: Stage IV T2N1MO. Regardless of tumor size, NETs metastasize in half of cases. Local excisions including endoscopic and laparoscopic ampullectomy were recommended only for poor surgical candidates. Ampullary NETs behave more aggressively than nonampullary NETs and their biological behaviors are irrespective of size. For patients of acceptable surgical risk, we recommend radical resection utilizing Whipple procedure.