Liver & intrahepatic bile ducts

Developmental anomalies / cysts

Polycystic liver disease / duct plate malformation



Last author update: 1 April 2012
Last staff update: 4 August 2022

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PubMed Search: Polycystic liver disease


Komal Arora, M.D.
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Cite this page: Arora K. Polycystic liver disease / duct plate malformation. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/liverpolycysticliverdisease.html. Accessed February 22nd, 2024.
Definition / general
  • 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+ versus < 5% in teenagers)
  • 1 - 7% risk of adenocarcinoma if coexisting Caroli disease; otherwise extremely rare
  • Complications: infection, cholangiocarcinoma, squamous cell carcinoma
Gross description
  • Multiple variably sized unilocular cysts, liver rarely is massively enlarged
Gross images

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Massive hepatomegaly and numerous cysts

Microscopic (histologic) description
  • 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
Microscopic (histologic) images

Contributed by Debra Zynger, M.D.

Low power

Cysts

von Meyenberg complex

Liver parenchyma

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