Ampullary Neuroendocrine Tumor in a Cirrhotic Patient without Neurofibromatosis: Case Report

Abstract

A 61 year old African American gentleman with cirrhosis secondary to Hepatitis C was referred for evaluation of an ampullary lesion. He presented with intermittent, epigastric abdominal pain with early satiety but no nausea, vomiting, or weight loss, and there was no personal or family history of neurofibromatosis. He underwent an EGD that revealed a 2 cm ampullary lesion, biopsies positive for low-grade NET. Somatostatin receptor scintigraphy as well as serum gastrin and chromogranin levels were normal. The patient underwent EUS which revealed a 2cm well-circumscribed lesion involving the major papilla, confined to mucosal and submucosal layers, with no intraductal extension. Next, using a side-viewing duodenoscope, a papillectomy including en bloc resection of the lesion was accomplished using salinelift snare-cautery from a caudal approach (Figure 1). Stents were placed in both the common bile and pancreatic ducts (Figure 2). Pathology of the mass revealed well-differentiated NET, positive for synaptophysin and chromogranin, Ki-67 index of 1%. Three weeks following the procedure, stents were removed and multiple random biopsies were taken to assess for remnant carcinoid tumor, which were negative. Ampullary neuroendocrine tumors are rare, comprising less than 2% of tumors of the ampulla of Vater. Due the rarity of these tumors, treatment protocols regarding ampullary neuroendocrine tumors are controversial. Endoscopic resection may be undertaken in those with smaller tumors that do not invade the muscularis propria and with less than one centimeter of intraductal extension. In potential candidates, endoscopic ultrasound is used for assessment of tumor stage, including depth of intraductal extension, which may then be followed by ERCP for endoscopic papillectomy and ductal stenting in those with amenable features. Several studies have suggested endoscopic resection of small periampullary NETs has similar outcomes as surgical resection but with significantly less morbidity. Surgical management is usually recommended for larger ampullary NETs and those with other features unfavorable for endoscopic resection. In those with amenable features or at increased risk for adverse events from surgery, such as the patient described above, endoscopic papillectomy represents a viable treatment modality for ampullary NETs.Figure 1Figure 2Figure 3