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Liver and intrahepatic bile ducts - Tumor

Benign tumors

Biliary papillomatosis


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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● Now known as intraductal papillary neoplasm (IPN) (WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 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 Caroliís disease, choledochal cyst, polyposis coli, hepatolithiasis and ulcerative colitis
● Most patients die within 3 years due to cholangitis and hepatic failure

Case reports
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● 66 year old man with cirrhosis due to Hepatitis C and malignant transformation (Arch Pathol Lab Med 2002;126:369)

Treatment
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● Difficult to treat because multifocal and propensity to grow and spread along the biliary tree
● Liver transplant may be helpful

Gross description
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● 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

Micro description
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● 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

Micro images
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Fibrovascular cores and invasive disease in case report above

End of Liver and intrahepatic bile ducts-nontumor > Benign tumors > Biliary papillomatosis


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