18 January 2007 - Case #70

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This case was contributed by Dr. Hanni Gulwani, Sir Ganga Ram Hospital, New Delhi, India.

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Case #70

Clinical history:
A 47 year old woman presented with pain in the right hypochondrium (upper lateral abdomen). CT scan showed a cystic space occupying lesion in the liver measuring 6 x 5 cm, which was excised.

Microscopic images:

Low power

High power

High power

High power

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Diagnosis: Biliary mucinous cystadenoma of liver

Stain images:


Progesterone receptor

Progesterone receptor

The low power images show a cystic, multiloculated lesion with adjacent normal liver (L). The high power images show a cyst lined by benign mucinous columnar epithelium. Image 7 shows ovarian type stroma. The epithelium is immunoreactive for CA19-9 and the stromal nuclei stain for progesterone receptor.

Biliary cystadenomas represent 5% of hepatic solitary cysts. Over 90% occur in women, with a mean age of 45 years. Although most (84%) are intrahepatic, they also occur within the common bile duct (6%), hepatic ducts (4%), cystic duct (4%) and gallbladder (2%). They may be associated with polycystic liver disease or abnormal hepatobiliary anatomy.

They are usually encapsulated, large (mean diameter: 15 cm), mucinous and multilocular, and may contain up to several liters of fluid. The inner lining is smooth, with few trabeculations or polypoid cystic projections. The presence of nodules of solid tissue suggests malignancy, which occurs in up to 25% of cases.

Histologically, they are lined by a single layer of columnar cuboidal mucinous epithelium with basal nuclei and apical mucin. Tumors in women have spindle cell ovarian type stroma, similar to that in pancreatic mucinous cystic neoplasms. The spindle cells may contain fat and smooth muscle. Although typically bland, there may be dysplastic foci. The uncommon serous tumors have clear, glycogen rich cytoplasm but no mucin or spindle cell stroma.

The epithelium in biliary cystadenomas is immunoreactive for cytokeratin, EMA, CA19-9 and CEA. The stromal cells are immunoreactive for muscle specific actin, vimentin and usually for estrogen and progesterone receptor (Dig Dis Sci 2006;51:623).

These tumors are usually slow growing and have a good prognosis after surgical excision.

The differential diagnosis includes borderline tumors (with high grade dysplasia and complex architecture) and invasive tumors, which must be ruled out by taking numerous sections.

References: World J Gastroenterol 2006;12:6062 (case report)

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