Biliary Cystadenoma and Cystadenocarcinoma
Matthew M. Yeh, MD, PhD
Key Facts
Clinical Issues
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Biliary cystadenoma almost exclusively occurs in women
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CA19-9 and CEA in cyst fluid helps differentiate between simple cyst and biliary cystic neoplasm
Macroscopic Features
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Solitary, multiloculated cystic neoplasm
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Clear, mucinous, or opalescent cystic fluid
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Cyst lining may be smooth, trabeculated, or have papillary excrescences
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Thickened, nodular areas suggest malignancy
Microscopic Pathology
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Similar to mucinous cystic neoplasm of pancreas
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Cystadenoma
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Lined by mucinous columnar epithelium with focal cuboidal, flattened, or papillary areas
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May have gastric or intestinal metaplasia
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Varying degrees of dysplasia may be present
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Densely cellular ovarian-like stroma positive for estrogen and progesterone receptors and inhibin
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Cystadenocarcinoma
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Most arise from preexisting cystadenoma
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Invasion of underlying stroma by malignant glands or single cells
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Top Differential Diagnoses
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Cystic variant of biliary intraductal papillary neoplasm
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Solitary bile duct cysts
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Ciliated hepatic foregut cyst
The classic spindled and cellular ovarian-type stroma is seen underneath the cyst lining of a biliary cystadenoma. |
This graphic shows a lobulated complex cystic mass with a vascularized wall, areas of solid growth, and well-defined septa typical of biliary cystadenocarcinoma. |
TERMINOLOGY
Definitions
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Cystic biliary neoplasm arising within liver
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May arise in extrahepatic biliary tree, including gallbladder
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ETIOLOGY/PATHOGENESIS
Unknown
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May arise from gallbladder precursor elements or peribiliary glands
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Most cystadenocarcinomas arise from preexisting biliary cystadenoma
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Some may represent cystic variant of cholangiocarcinoma
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Occasionally may arise in biliary cysts
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CLINICAL ISSUES
Epidemiology
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Incidence
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Rare; < 5% of cystic lesions of liver
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Age
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Average is 40-50 years
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Gender
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Biliary cystadenoma almost exclusively occurs in women
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Biliary cystadenocarcinoma may be seen in men, given varied pathogenesis
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Presentation
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Pain, mass, and occasionally jaundice
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Some patients are asymptomatic
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Laboratory Tests
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CA19-9 and CEA in cyst fluid helps differentiate between simple cyst and biliary cystic neoplasm
Treatment
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Surgical approaches
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Complete resection
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Prognosis
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Surgical resection should be curative
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Incompletely resected tumor may recur or undergo malignant transformation
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IMAGE FINDINGS
Ultrasonographic Findings
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