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Extrahepatic bile ducts

Tumors

Intraductal papillary neoplasms of biliary tract


Reviewer: Hanni Gulwani, M.D. (see Reviewers page)
Revised: 16 February 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Uncommon
● 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
● Risk factor for cholangiocarcinoma, biliary obstruction, recurring ascending cholangitis
● Are often carcinomas (HPB (Oxford) 2009;11:684)
● p21, p53, cyclin D1, DPC4 are involved in neoplastic progression (Hum Pathol 2008;39:1153)

Case reports
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● 67 year old woman with polypoid mass (Pathol Int 2010;60:516)

Gross description
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● Either polypoid, cast-like growth, superficial-spreading, or cystic type, but does not correlate with prognosis (Abdom Imaging 2011;36:438)

Micro description
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● Papillary fronds with fine vascular cores
● Epithelial cells are either biliary type or have gastric or intestinal differentiation with goblet cells and Paneth cells
● Production of extracellular intraductal mucin less common than papillary IPMN
● Variants have oncocytic changes or cysts (Mod Pathol 2006;19:1243)
Borderline tumor: mild to moderate nuclear atypia and nuclear pseudostratification limited to basal 2/3 of the epithelium
Carcinoma: severe cytological atypia, loss of nuclear polarity, or architectural cribriforming/papillary fusion is present

Micro images
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Various images

Positive stains
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● MUC5AC, MUC6, Claudin 18 and HepPar1
● Variable CK20 (Hum Pathol 2002;33:503)
● Tumors with oncocytic changes have gastric phenotype (Hum Pathol 2009;40:1543), express Wnt pathway proteins beta-catenin and E-cadherin (Pathology 2007;39:413)

Negative stains
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● p53 (Hum Pathol 2003;34:902)

Molecular description
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● Kras activating mutations (29%), 18q- (31%) but no loss of DPC4
● Often has microsatellite instability (Mod Pathol 2002;15:1309)

End of Extrahepatic bile ducts > Tumors > Intraductal papillary neoplasms of biliary tract


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