Gallbladder & extrahepatic bile ducts

Extrahepatic bile duct tumors

Intraductal papillary neoplasm of biliary tract

Last author update: 1 September 2012
Last staff update: 24 June 2022

Copyright: 2003-2022,, Inc.

PubMed Search: Intraductal papillary neoplasms of biliary tract

Hanni Gulwani, M.B.B.S.
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Cite this page: Gulwani H. Intraductal papillary neoplasm of biliary tract. website. Accessed July 5th, 2022.
Definition / general
  • 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)
Clinical features
  • Preinavsive neoplasms at least 1 cm (Am J Surg Pathol 2012;36:1279)
  • Analogous to pancreatic (IPMN) and biliary counterparts
  • Show variable cellular lineages with a spectrum of dysplasia, mixture of papillary or tubular growth patterns
  • Relatively indolent behavior - better prognosis than pancreatobiliary type gallbladder carcinoma
Case reports
Gross description
  • Either polypoid, cast-like growth, superficial spreading or cystic type but does not correlate with prognosis (Abdom Imaging 2011;36:438)
Gross images

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Hilar tumor whose
connective tissue
contains multiple

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

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Immunostains: CK7+, MUC1+, MUC6+, MUC5AC-

Various cysts

Positive stains
Negative stains
Molecular / cytogenetics description
  • KRAS activating mutations (29%), 18q- (31%) but no loss of DPC4
  • Often has microsatellite instability (Mod Pathol 2002;15:1309)
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