Revision 6 for 'Intraductal papillary neoplasm of the bile duct'

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Intraductal papillary neoplasm of the bile duct

Intraductal papillary neoplasm of the bile duct (IPNB) is a pre-invasive biliary tree neoplasm considered to be a precursor of cholangiocarcinoma

Terminology

Biliary papillary adenoma and noninvasive papillary carcinoma of the biliary tract were terms used to refer to localized low-grade and high-grade IPNBs 1. Whereas biliary papillomatosis was used to describe what is now known as diffuse IPNBs 1

Epidemiology

IPNBs have been reported to be more frequent in Asia, in regions where hepatolithiasis and clonorchiasis are endemic 2,6. They account for 10 to 30% of all bile duct tumors in countries such as Japan, China, and Korea, compared to ~9% in Western countries 2

Other risk factors for IPNBs 2:

Clinical presentation

Reported symptoms include abdominal pain and jaundice. 

Pathology

IPNB is a counterpart for the intraductal papillary mucinous neoplasm of the pancreas (IPMN), with both showing intraluminal growth, same histological subtypes, and the association with mucin hypersecretion 1-4

Location

Although they potentially affect any segment of the biliary tree, IPNBs are more frequently found involving the intrahepatic ducts or at the hepatic hilum and, for unclear reasons, most commonly within the left-sided ducts 2

Macroscopic appearances

On gross specimens, they present as solitary or multiple papillary lesions within a dilated bile duct 2. Different from pancreatic IPMN, only in about one-third of IPNB cases show mucin at a macroscopic level 1

Microscopic appearance

Different from the pancreatic IPMN, which are commonly lined by mucinous epithelium, IPNBs are lined by biliary epithelium 4. There is a papillary proliferation of the atypical epithelial cells with central fibrovascular cores, as well as mucinous cells 1,2

Different from biliary intraepithelial neoplasia, IPNB is divided into two groups based on the degree of dysplasia: low or intermediate-grade dysplasia and high-grade dysplasia 1

Histological subtypes are 1-6

  • pancreatobiliary
    • more frequent in Western countries 1
    • frequently associated with 1
      • high-grade dysplasia or invasive carcinoma
      • expression of the mucin core protein MUC1
  • intestinal
    • more frequent in Asia countries 1
    • frequently associated with a large amount of mucin production 
  • gastric
  • oncocytic 

History and etymology

Biliary papillomatosis was initially described in 1894 by Chappet. In 1959 anatomical description was given by Caroli.

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