Cholangiocarcinoma occurring in a liver with multiple bile duct hamartomas (von Meyenburg complexes)

Arch Pathol Lab Med. 2000 Nov;124(11):1704-6. doi: 10.5858/2000-124-1704-COIALW.


A 59-year-old woman presented with epigastric pain and weight loss. Ultrasound, computed tomography, and magnetic resonance imaging scans of the abdomen showed a tumor in segments 6 and 7 of the right liver lobe, measuring 8 cm in greatest diameter. The tumor was subsequently resected, and histopathology showed a poorly differentiated adenocarcinoma immunoreactive for CA 19-9 and cytokeratin 19. In the absence of any other clinically detectable primary tumor, the lesion was diagnosed as a peripheral intrahepatic cholangiocarcinoma. In addition, multiple bile duct hamartomas were found in the surrounding parenchyma. The tumor was unrelated to Caroli disease, primary sclerosing cholangitis, ulcerative colitis, or nonbiliary cirrhosis, as demonstrated by further clinical and histopathologic investigations, but probably was associated with the presence of multiple bile duct hamartomas. To our knowledge, this is the eighth reported case of a cholangiocarcinoma associated with multiple bile duct hamartomas.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Bile Duct Neoplasms / metabolism
  • Bile Duct Neoplasms / pathology*
  • Bile Ducts, Intrahepatic / chemistry
  • Bile Ducts, Intrahepatic / pathology*
  • CA-19-9 Antigen / analysis
  • Cholangiocarcinoma / metabolism
  • Cholangiocarcinoma / pathology*
  • Female
  • Hamartoma / pathology*
  • Humans
  • Immunohistochemistry
  • Keratins / analysis
  • Liver Neoplasms / metabolism
  • Liver Neoplasms / pathology*
  • Male
  • Middle Aged


  • CA-19-9 Antigen
  • Keratins