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Liver and intrahepatic bile ducts - Tumor

Benign tumors

Biliary cystadenoma


Reviewers: Shilpa Jain, M.D. (see Reviewers page)
Revised: 16 April 2014, last major update July 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Less than 5% of all hepatic solitary cysts
● 95% occur in women, mean age 45 years (range 2-87 years)
● 84% are intrahepatic, also in common bile duct (6%), hepatic ducts (4%), cystic duct (4%), gallbladder (2%)
● Single and multiloculated with predilection for right lobe of the liver
● Associated with polycystic liver disease, abnormal hepatobiliary anatomy
● Usually slow growing with good prognosis after surgical excision, although 25% have coexisting malignancy
Complications: intracystic hemorrhage, bacterial infection, spontaneous rupture, recurrence, malignant transformation
Laboratory: elevated CA 19-9 (in cases with ovarian type stroma) and CEA in cyst fluid and serum

Radiology description
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● Calcification in 20% (resemble echinococcal cyst)

Treatment
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● Complete excision (rarely has delayed recurrence)

Case reports
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● 32 year old woman with tumor arising from left hepatic duct (Arch Pathol Lab Med 2001;125:1507)
● 47 year old woman (Case of the Week #70)
● 59 year old woman with hepatic smooth muscle tumor encasing hepatobiliary cystadenoma (Am J Surg Pathol 1999;23:854)

Gross description
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● Encapsulated, solitary, mean 15 cm (range 3-30 cm), usually mucinous, multilocular by definition (locules have varied sizes)
● Contains up to several liters of fluid
● Smooth inner surface with few trabeculations or polypoid cystic projections
● Rarely contains gallstones
● Nodules of solid tissue suggests malignancy

Gross images
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Large, smooth lined cyst


Contributed by: Dr. Nasir Uddin, The Aga Khan University Hospital (Pakistan)

Micro description
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● Usually mucinous: lined by single layer of columnar-cuboidal mucinous epithelium with basal nuclei and apical mucin
● 3 types: (a) hepatobiliary cystadenoma with mesenchymal stroma, (b) without mesenchymal stroma, and (c) intraductal polypoid with mesenchymal stroma
● Spindle-cell ovarian type stroma only in women (resembles pancreatic mucinous cystic neoplasms)
● Spindle cells may contain fat and smooth muscle
● May have collagenous zone above stroma (resembling collagenous colitis)
● Capsule composed of dense collagen with blood vessels, variable bile ducts
● May have squamous or intestinal metaplasia, often neuroendocrine cells; may have dysplastic or borderline foci
● May have ulceration with macrophages containing lipofuscin or hemosiderin, cholesterol clefts with foreign body giant cell reaction or calcification
● No/rare atypia, no/rare mitotic figures; no communication with the bile ducts
Serous - lined by bland, flat to cuboidal cells with clear, glycogen-rich cytoplasm, no spindle cell stroma; no mucin; may represent hepatic metastasis from pancreatic serous cystadenocarcinoma

Micro images
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Case of Week


Contributed by: Dr. Nasir Uddin, The Aga Khan University Hospital (Pakistan)

Positive stains
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Epithelial cells: cytokeratin, EMA, CEA, CA19-9
Stromal cells: muscle specific actin, vimentin; usually ER and PR (Dig Dis Sci 2006;51:623)

Differential diagnosis
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● Borderline tumor: high grade dysplasia and complex architecture
● Invasive tumor: put through numerous sections to exclude invasion
● Foregut/ciliated cyst: asymptomatic, males, unilocular, present in segment IV, ciliated columnar/cuboidal epithelial lining with subepithelial connective tissue surrounded by a muscle layer

End of Liver and intrahepatic bile ducts - Tumor > Benign tumors > Biliary cystadenoma


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