Extrahepatic bile ducts
Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 15 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
● 0.3% of tumors in extrahepatic bile ducts
● 2/3 women, mean 50 years old, range 37-67 years
● No systemic endocrine symptoms
● Rarely associated with MEN1 and von Hippel-Lindau syndrome
● Indolent course with long survival even if nodal or hepatic metastases (Am J Surg Pathol 2000;24:1501)
● Tumor size (>2 cm) appears to be the best predictor of aggressive behavior (Arch Pathol Lab Med 2010;134:1075)
● 31 year old woman with VHL
(World J Gastroenterol 2008;14:1299)
● 60 year old woman with malignant carcinoid tumor of the cystic duct (Hepatobiliary Pancreat Dis Int 2009;8:640)
● 73 year old man with clear cell carcinoid tumor of distal common bile duct (World J Surg Oncol 2007;5:6)
Clear cell carcinoid tumor of the distal common bile duct: golden yellowish polypoid mass
● Nests, cords or trabeculae of small cells with granular chromatin
● Perineural and vascular invasion is common
31 year old woman with von Hippel-Lindau syndrome
● Chromogranin, synaptophysin, serotonin, loss of DPC4
Electron microscopy description
● Numerous membrane bound, round, neurosecretory granules
End of Extrahepatic bile ducts > Tumors > Carcinoid tumor
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