Liver and intrahepatic bile ducts-nontumor

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Liver and intrahepatic bile ducts-nontumor

Developmental anomalies/cysts

Polycystic liver disease

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 23 April 2012, last major update April 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Autosomal dominant, associated with autosomal dominant (but not autosomal recessive) polycystic kidney disease (71-93%) and defect in ADPKD1 gene on #16
● Cysts don’t communicate with biliary tree
● 80% occur in females
● Associated with abdominal tenderness, pain with stooping; may present during pregnancy
● Cysts more common with increased age (75% at age 70+ vs. <5% in teenagers)
● 1-7% risk of adenocarcinoma if coexisting Caroli’s disease; otherwise extremely rare
● Complications: infection, cholangiocarcinoma, squamous cell carcinoma

Gross description
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● Multiple variably sized unilocular cysts, liver rarely is massively enlarged

Gross images
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Massive hepatomegaly and numerous cysts with hemorrhage

Massive hepatomegaly and numerous cysts

Micro description
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● Multiple diffuse cystic lesions resembling solitary cysts, lined by cuboidal to flat biliary epithelium, containing straw colored fluid
● 40% have identifiable von Meyenburg complexes
● Don’t contain pigmented material

Micro images
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Cystic lesions with von Meyenburg complexes

Cyst wall epithelium

Additional references
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● Gastroenterology 2011;140:1855, Aliment Pharmacol Ther 2011;34:702

End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Polycystic liver disease

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