Liver and intrahepatic bile ducts - tumor - Biliary cystadenocarcinoma

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Liver and intrahepatic bile ducts - tumor

Other malignancies

Biliary cystadenocarcinoma

Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.

General
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● 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
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● 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 intraabdominal 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

Radiology images
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MRI: multiple cysts and solid lesion

Case reports
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● 43 year old woman with tumor exhibiting oncocytic differentiation (Arch Pathol Lab Med 2004;128:e25)
● 71 year old man with multiple tumors resembling oncocytes (Mod Pathol 2001;14:1304)

Gross description
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● 5-20 cm cysts, may have blood tinged fluid, solid areas and large papillary masses

Gross images
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Multiple cysts and solid lesion

Micro description
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● 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; woman also have features of cystadenoma
● Rarely adenosquamous, hepatoid or oncocyte-like features; malignant diagnosis is based on stromal invasion

Micro images
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Oncocytic tumor

Papillary oncocytic tumor within fibrous cyst wall

Various images

Positive stains
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● CK7, AE1/AE3, HepPar1; variable CEA and C19-9

Negative stains
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● CK20, mucin, AFP, calretinin, CD31 and chromogranin

Electron microscopy description
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● Numerous mitochondria

Differential diagnosis
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● Metastases from pancreas, ovary and appendix

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Biliary cystadenocarcinoma

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