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


Topic Completed: 1 April 2012

Revised: 4 February 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Polycystic liver disease[TI] free full text[sb]


Komal Arora, M.D.
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Cite this page: Arora K. Polycystic liver disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/liverpolycysticliverdisease.html. Accessed February 18th, 2020.
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+ vs. < 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

Images hosted on other servers:

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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