Abstract

BACKGROUND
Primary neuroendocrine tumors (NET) of the extrahepatic biliary (EB) tree are a rare entity, with less than 100 reported cases in the literature.
CASE REPORT
A 42-year-old woman was admitted to the Emergency Department of our hospital with a 3-week history of itching of the hands, forearms, and lower legs, followed by symptoms of obstructive jaundice. Abnormal initial laboratory test results showed hyperbilirubinemia and elevated liver transaminases. Based on abdominal ultrasonography, choledocholithiasis was suspected. Subsequent magnetic resonance imaging of the hepatobiliary system showed suspected Mirizzi syndrome vs biliary neoplasm of the proximal common bile duct. Abdominal computed tomography showed cholestasis, leading to suspected choledocholithiasis vs cholangiocarcinoma (type-1). An endoscopic retrograde cholangiopancreatography was performed with biliary and pancreatic duct stenting for biliary transduodenal drainage, and brush cytology was obtained from the biliary stricture. A brush cytology sample was positive for adenocarcinoma cells. A multidisciplinary consilium referred the patient to surgical resection of the bile duct tumor. The extrahepatic bile duct resection with cholecystectomy en bloc, lymphadenectomy from the hepatoduodenal ligament, Roux-en-Y anastomosis, and biliary drainage was performed. Histopathology of the resected specimen revealed a neuroendocrine carcinoma. A multidisciplinary team referred the patient to adjuvant systemic treatment consisting of 8 cycles of FOLFOX6 treatment. After chemotherapy course, there was no evidence of disease relapse.
CONCLUSIONS
This case highlights the utmost importance of multidisciplinary teamwork. Because of the rarity of extrahepatic bile duct NET, preoperative diagnosis is challenging because of an unusual lack of symptoms; therefore, histological examination is required for definitive diagnosis.

Keywords: Case Reports; Pruritus; Cholangiopancreatography, Endoscopic Retrograde