1365 A Rare Case of Neuroendocrine Tumor Originating in Liver Diagnosed by ERCP Using a Spyglass Direct Visualization System

Abstract

INTRODUCTION: Neuroendocrine carcinoma is predominantly found in the gastrointestinal tract (54-75%) with 44%, 19%, 16%, 10% and 7% in the small intestines, rectum, appendix, colon and stomach respectively. Primary liver neuroendocrine carcinoma comprises around 0.3% of all neuroendocrine tumors. This is a case of a neuroendocrine carcinoma with a focus of cholangiocarcinoma presenting as a biliary stricture diagnosed by ERCP using the spyglass visualization system. CASE DESCRIPTION/METHODS: A 75 year old female with a prior history of rheumatoid arthritis presented with persistent nausea, vomiting, diarrhea and abdominal pain and a 20 lb weight loss since December 2017. An ultrasound revealed intrahepatic biliary ductal dilation in the left lobe with no visible mass and a coarse hepatic echotexture. A subsequent MRI of the abdomen demonstrated left lobe intrahepatic biliary ductal dilation with a hypointense 1 cm region within the liver parenchyma associated with a biliary stricture. The biliary stricture in the left hepatic branch was visualized with the spyglass direct visualization cholangioscope introduced through an ERCP scope. Biopsies obtained through the cholangioscope revealed high grade dysplasia. The patient underwent a left lobectomy of liver with hepatic artery and portal lymph node dissection. Lymph nodes were negative for malignancy. The biliary stricture was from a high grade neuroendocrine carcinoma with a microscopic focus of cholangiocarcinoma with extensive lymphovascular and perineural invasion. The margin of the bile duct and hepatic hilum were involved by neuroendocrine carcinoma. Post operative PET scan showed a small focus of activity without any signs of distant metastasis. DISCUSSION: SpyGlass direct visualization system is a relatively new technique in ERCP. Recently published prospective data confirm that the overall success rates for adequate tissue sampling is nearly 90%. ERCP has limitations for sampling lesions in the bile duct without direct visualization. Conventional ERCP biliary sampling yields are 20% to 30% for cytology brushing to approximately 50% with a combined brush/standard biopsy forceps approach. If sampling is negative, the choice is either radical surgery or continual observation with an MRI. Nowadays, ERCP with spyglass technique and targeted direct visualization biopsy affords vastly higher yields. Direct visualization is now the gold standard to obtain biopsy samples in cases of biliary strictures.