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Liver and intrahepatic bile ducts - Tumor

Benign tumors

Intraductal papillary neoplasms of biliary tract


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● Uncommon tumor arising from peribiliary glands (AJR Am J Roentgenol 2011;197:1111)
● Solitary or may spread along biliary tree to cystic duct or duodenal papilla
● May resemble intrapapillary mucinous neoplasms of pancreas as both arise within a dilated duct system and demonstrate predominantly intraductal growth
● Associated with Clonorchis sinensis infection (Virchows Arch 2008;453:589)
● Often are carcinomas
● Risk factor for cholangiocarcinoma, biliary obstruction, recurring ascending cholangitis

Micro description
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● Papillary fronds with fine vascular cores
● Epithelial cells are either pancreaticobiliary type, have gastric or intestinal differentiation with goblet cells and Paneth cells, or have oncocytic cells (Hum Pathol 2009;40:1543)
● Production of extracellular intraductal mucin less common than pancreatic IPMNs
● However, mucin-producing IPMN of bile ducts show striking similarities to pancreatic IPMN and nonmucin producing IPM of bile ducts is heterogenous disease (Am J Surg Pathol 2011;35:512)
Borderline tumors: mild to moderate nuclear atypia and nuclear pseudostratification limited to basal 2/3 of the epithelium
Carcinomas: severe cytological atypia, loss of nuclear polarity or architectural cribriforming / papillary fusion is present

Positive stains
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● Claudin18 (Virchows Arch 2011;459:73)
● Pancreatobiliary: MUC1, MUC5AC, MUC6
● Intestinal: MUC2, CDX2, MUC5AC
● Gastric: MUC5AC
● Oncocytic: MUC5AC, MUC6

Negative stains
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● p53, CK20, MUC2 (except in intestinal type, Hum Pathol 2002;33:503)

Molecular description
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● KRAS activating mutations (29%), 18q- (31%), no loss of DPC4 (Hum Pathol 2003;34:902)

End of Liver and intrahepatic bile ducts - Tumor > Benign tumors > Intraductal papillary neoplasms of biliary tract


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