Carcinoid tumor of the biliary tract: Treating a rare cause of bile duct obstruction

Biliary carcinoids are rare with fewer than 30 cases reported in the English literature. The objective of this report is to describe an additional patient found to have a biliary carcinoid and to define the presentation, diagnosis, and management of patients with this rare biliary tumor. In our case the patient initially presented with clinical jaundice and elevated transaminases. Endoscopic retrograde cholangiogram established a mass suspicious for cholangiocarcinoma (Klatskin tumor). The patient was initially managed with an endostent, which was later removed in favor of a percutaneous transhepatic cholangiogram tube. At the time of surgery successful removal of a firm nodular mass at the area of the ductal bifurcation was achieved and biliary continuity re-established with a Roux-en-Y hepaticojejunostomy. Pathology revealed carcinoid tumor of the bile duct with one lymph node positive for tumor. The patient did not receive any adjuvant radiation or chemotherapy. This case serves to highlight that extrahepatic biliary carcinoids constitute a rare but identifiable subset of bile duct tumors. Diagnostic workup should include US, CT, and cholangiography. Surgical exploration is universally indicated in physiologically fit patients with operative management to include resection and re-establishment of biliary continuity. Data on adjuvant therapy remain investigational; however, available information suggests that patients with biliary carcinoid have an overall favorable prognosis after aggressive surgical management.