Disorders of the Intra- and Extrahepatic Bile Ducts
Case 8 -
Polycystic Liver Disease, With Autosomal Dominant Polycystic Renal Disease
Kay Washington, M.D., Ph.D.
Pathology, C-3321 MCN
Vanderbilt University Medical Center
Nashville, TN 37232-2561
This 45 year old woman developed abdominal pain, post-prandial fullness and lower extremity
edema over several months. Physical exam revealed marked hepatomegaly. A CT scan of the abdomen
revealed near-total replacement of the right lobe of the liver by cystic lesions and several large cysts
in the left lobe. The kidneys also contained multiple cysts. The right lobe of the liver was resected.
The resected portion of the liver was distorted by cysts ranging in size from
0.5 cm to 4 cm. The larger cysts contained clear lightly bile stained fluid. Microscopically the cysts
are lined by a simple cuboidal epithelium that resembles biliary epithelium. Scattered von Meyenburg
complexes are present in the adjacent liver and in the fibrous stroma between cysts.
Click on each slide thumbnail image for an enlarged view:
Slide 19 Polycystic Liver Disease Multiple unilocular cysts of varying sizes are found in the liver in polycystic liver disease. Biliary hamartomas, or von Meyenburg complexes, are frequently found in the vicinity of the cysts and probably give rise to them by progressive accumulation of fluid.
Slide 20 Polycystic Liver Disease The cysts of autosomal dominant polycystic disease involving the liver are lined by a simple cuboidal to low columnar biliary-type epithelium.
Diagnosis: Polycystic Liver Disease, With Autosomal Dominant Polycystic Renal Disease