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

Clinical History 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. Morphologic Findings 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