Biliary intraductal papillary mucinous neoplasia: three case reports

Virchows Arch. 2009 May;454(5):589-94. doi: 10.1007/s00428-009-0767-1. Epub 2009 Apr 4.

Abstract

Intrahepatic cholangiocarcinoma is subdivided as mass-forming, periductal-infiltrating, and intraductal-growing types. Intraductal-growing type is an entity described in recent years as mucin-producing intrahepatic cholangiocarcinoma or intrahepatic (biliary) intraductal papillary mucinous neoplasia (b-IPMN). b-IPMN is classified as adenoma, borderline tumor, carcinoma in situ, and carcinoma, from benign to malignant. Using a different classification, b-IPMNs are subdivided into intestinal, pancreatobiliary, gastric, or oncocytic based on morphology of the cells forming the lesion and expression of MUC1, MUC2, and MUC5 gene proteins in the mucin family. The clinical and histopathological features of b-IPMN diagnosed in three cases are presented herein. Case 1 was classified as borderline. Case 2 was diagnosed as carcinoma in situ. Case 3 had large invasive areas, and was diagnosed as carcinoma. In all three cases, immunohistochemical investigation revealed MUC1 and MUC5AC to be positive, and MUC2 to be negative. We present herein three cases diagnosed with the clinical and pathological findings of a new entity in the literature, b-IPMN, and we discuss the macroscopic, histological, and immunohistochemical features.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma, Mucinous / metabolism
  • Adenocarcinoma, Mucinous / pathology*
  • Adenocarcinoma, Mucinous / surgery
  • Aged
  • Bile Duct Neoplasms / metabolism
  • Bile Duct Neoplasms / pathology*
  • Bile Duct Neoplasms / surgery
  • Bile Ducts, Intrahepatic / metabolism
  • Bile Ducts, Intrahepatic / pathology*
  • Biomarkers, Tumor / metabolism
  • Carcinoma in Situ / metabolism
  • Carcinoma in Situ / pathology
  • Carcinoma in Situ / surgery
  • Carcinoma, Papillary / metabolism
  • Carcinoma, Papillary / pathology*
  • Carcinoma, Papillary / surgery
  • Cholangiocarcinoma / metabolism
  • Cholangiocarcinoma / pathology*
  • Cholangiocarcinoma / surgery
  • Disease-Free Survival
  • Hepatitis B / complications
  • Hepatitis B / surgery
  • Humans
  • Immunoenzyme Techniques
  • Liver Cirrhosis / surgery
  • Liver Cirrhosis / virology
  • Liver Transplantation
  • Male
  • Middle Aged
  • Mucin 5AC / metabolism
  • Mucin-1 / metabolism
  • Pancreatic Neoplasms / metabolism
  • Pancreatic Neoplasms / pathology
  • Pancreatic Neoplasms / surgery
  • Treatment Outcome

Substances

  • Biomarkers, Tumor
  • MUC1 protein, human
  • MUC5AC protein, human
  • Mucin 5AC
  • Mucin-1