Liver and intrahepatic bile ducts - tumor
Benign tumors / conditions
Biliary papillomatosis

Author: Deepali Jain, M.D. (see Authors page)

Revised: 15 November 2017, last major update February 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Biliary papillomatosis[TI]

Cite this page: Jain, D. Biliary papillomatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/livertumorbiliarypap.html. Accessed December 11th, 2017.
Definition / general
  • Now known as intraductal papillary neoplasm (IPN) (Bosman: WHO Classification of Tumours of the Digestive System, 4th Edition, 2010)
  • Rare, 50 cases reported
  • 2/3 men, usually ages 60+ years in intrahepatic bile ducts, hilar and extrahepatic bile ducts
  • Synchronous and dyssynchronous disease can develop in intrahepatic and extrahepatic biliary tree, gallbladder, major pancreatic ducts
  • Multiple papillary adenomas extensively throughout intra or extrahepatic biliary tract
  • Often recurs, 25% have malignant transformation but only rare metastases (to lung)
  • Patients may present with recurrent cholangitis and obstructive jaundice
  • Associated with Caroli disease, choledochal cyst, polyposis coli, hepatolithiasis and ulcerative colitis
  • Most patients die within 3 years due to cholangitis and hepatic failure
Case reports
Treatment
  • Difficult to treat because multifocal and propensity to grow and spread along the biliary tree
  • Liver transplant may be helpful
Gross description
  • Focal (localized), multifocal or diffuse
  • Dilated bile ducts are fusiform or cystic (unilocular or multilocular)
  • Inner surface of ducts has velvety finger like papillary growths with masses filling dilated ducts
  • Masses are soft, friable, white red tan
  • 1/3 secrete mucin in the duct lumen, accounting for prior terminology of mucin secreting biliary tumor
Microscopic (histologic) description
  • 4 subtypes: pancreatobiliary, intestinal, gastric, oncocytic; based on morphology and mucin expression (Am J Surg Pathol 2004;28:327, Hum Pathol 2009;40:1543)
  • Dilated ducts contain multiple papillary tumors composed of fibrovascular cores lined by columnar, pseudostratified, biliary type cells with numerous cytoplasmic mucin vacuoles; tumor may be solid or cribriform; varying cytologic atypia and mitotic activity
  • May have associated tubular adenocarcinoma with invasion
  • Also classified as IPN low grade and IPN high grade based on the highest degree of architectural and cytological atypia
  • Identify stromal invasion by thorough sampling
Microscopic (histologic) images

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Fibrovascular cores
and invasive
disease in case
report above