Liver and intrahepatic bile ducts - tumor
Other malignancies
Biliary cystadenocarcinoma

Author: Deepali Jain, M.D. (see Authors page)

Revised: 4 December 2017, last major update February 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Biliary cystadenocarcinoma[TI] liver

Cite this page: Jain, D. Biliary cystadenocarcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/livertumorbiliarycystadenocarcinoma.html. Accessed December 15th, 2017.
Definition / general
  • Rare cystic neoplasm of biliary origin; < 100 cases reported; < 5% of all intrahepatic tumors
  • Cysts represent dilated intrahepatic bile ducts invaded by tumor cells with a colloid carcinoma-like pattern, such as IPMN of pancreas
  • May be associated with intraductal oncocytic papillary neoplasm
Clinical features
  • Usually ages 50+ years; equal gender frequency
  • Usually mucinous, often associated with cystadenoma
  • Tumors more indolent in women with ovarian-like stroma, more aggressive in men
  • May have intra-abdominal dissemination as terminal event
  • Clinical differentiation from cystadenoma: older age, male gender, shorter symptom duration are suggestive of biliary cystadenocarcinoma
  • 50% survival at 4 years, better prognosis if spindle cell stroma
Case reports
Gross description
  • 5 - 20 cm cysts, may have blood tinged fluid, solid areas and large papillary masses
Gross images

Images hosted on other servers:

Multiple cysts and solid lesion

Microscopic (histologic) description
  • Papillary or tubulopapillary neoplasm of malignant cells lining fibrovascular cores that project into cystic cavities
  • Epithelium is columnar or cuboidal with stratification, loss of polarity, nuclear pleomorphism, prominent nucleoli and mitotic activity
  • Women may have ovarian-like stroma (not found in men); extensive stromal and hepatic parenchymal invasion; women also have features of cystadenoma
  • Rarely adenosquamous, hepatoid or oncocyte-like features; malignant diagnosis is based on stromal invasion
Microscopic (histologic) images

Images hosted on other servers:

Oncocytic tumor

Various images

Positive stains
Negative stains
Electron microscopy description
  • Numerous mitochondria
Differential diagnosis