Liver & intrahepatic bile ducts
Benign / nonneoplastic
Intraductal papillary neoplasm of the bile ducts


Topic Completed: 19 September 2019

Minor changes: 1 August 2020

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Intraductal papillary neoplasm of the bile ducts [title]

Zachary Dong, M.D.
Kimberley J. Evason, M.D., Ph.D.
Page views in 2019: 1,590
Page views in 2020 to date: 1,437
Cite this page: Dong Z, Evason KJ. Intraductal papillary neoplasm of the bile ducts. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livertumorintraductalpap.html. Accessed August 8th, 2020.
Definition / general
  • Papillary neoplasm consisting of fibrovascular stalks within cystically dilated intrahepatic bile ducts
  • WHO classification
    • Premalignant = intraductal papillary neoplasm with low grade, intermediate grade or high grade intraepithelial neoplasia
    • Malignant = intraductal papillary neoplasm with associated invasive carcinoma
Essential features
  • Papillary neoplasm within dilated intrahepatic bile duct(s)
  • Lacks ovarian stroma
  • High rate of associated invasive carcinoma (up to 74%) (Hepatology 2012;56:1352)
Terminology
  • Biliary papillomatosis
  • Biliary papilloma
Epidemiology
Sites
  • Intrahepatic bile ducts
  • Similar lesions can involve extrahepatic bile ducts, pancreas or gallbladder
  • Often multifocal
Pathophysiology
  • Stepwise progression from low grade dysplasia to high grade dysplasia to invasive carcinoma involving inactivation of TP53 and p16 (early), activating mutations in KRAS (early) and loss of SMAD4/DPC4 (late) (Mod Pathol 2014;27:73)
Etiology
Clinical features
  • Symptoms resulting from biliary obstruction = jaundice, abdominal pain
Diagnosis
  • Imaging (CT, MRI, ultrasound)
  • Surgical resection - extensive histologic sampling required to exclude invasive component
Laboratory
  • May have elevated bilirubin and alkaline phosphatase due to biliary obstruction
Radiology description
Radiology images

Images hosted on other servers:
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Dilated duct

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Papillary projections

Prognostic factors
Case reports
Treatment
  • Surgical resection is first line treatment
  • Palliative approaches include chemotherapy and laser ablation
Gross description
  • Soft, friable, villiform mass(es)
  • May be pedunculated, sessile or polypoid
Gross images

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Friable, villiform projections

Microscopic (histologic) description
  • Papillary neoplasm with complex tubulovillous or micropapillary architecture
  • Grows within dilated bile ducts
  • Lacks ovarian stroma
  • May form large, multilocular cysts with thin fibrous septations
  • Epithelium may be pancreaticobiliary (most common), intestinal, gastric or oncocytic
Microscopic (histologic) images

Contributed by Zachary Dong, M.D.
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Friable papillary projections

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Papillary lesion

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Pancreaticobiliary type epithelium

Cytology description
  • Hypercellular, with broad and double layered sheets of columnar epithelial cells and complex, three dimensional branching papillary configurations
Negative stains
  • ER / PR staining is negative in stroma cells (unlike mucinous cystic neoplasm)
  • SMAD4 / DPC4 staining may be lost in invasive component
Sample pathology report
  • Liver, left lobe, lobectomy:
    • Intraductal papillary neoplasm with low and high grade dysplasia, involving left hepatic duct
    • No invasive carcinoma
Differential diagnosis
  • Mucinous cystic neoplasm
    • Also a cystic hepatic lesion that may be lined by biliary type epithelium
    • Has ovarian type stroma and does not communicate with biliary lumen
  • Cholangiocarcinoma
  • Simple cyst, simple hepatic cyst, simple biliary cyst, bile duct cyst
    • Lacks papillary projections
Board review style question #1

    A 72 year old man presented with jaundice. CT scan showed dilatation of the left bile duct, which contained a papillary lesion. An image from the resection specimen is shown. Which of the following is true about this entity?

  1. The epithelium is most commonly intestinal type with goblet cells
  2. It lacks ovarian stroma
  3. It is almost always unifocal
  4. There is a strong male predominance
  5. There is a very low risk of associated invasive carcinoma
Board review answer #1
B. It lacks ovarian stroma (this is an intraductal papillary neoplasm)

Reference: Intraductal papillary neoplasm of the bile ducts

Comment Here
Board review style question #2
    Intraductal papillary neoplasms of the liver most often have which type of epithelium?

  1. Intestinal
  2. Oncocytic
  3. Pancreaticobiliary
  4. Ovarian
  5. Gastric
Board review answer #2
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