Sections from the liver show extensive cyst formation affecting more than 90% of the liver parenchyma. The limited amount of liver tissue which remains shows a variety of changes varying from atrophy to hemorrhage. Similar epithelium lined cysts are seen in the kidney. Some cysts have ruptured and lead to hemorrhagic necrosis, calcification and fibrosis. Focal cholesterol clefts and foreign body giant cells are seen. The spleen shows an expanded red pulp and multiple cystic spaces lined by flattened cells.