Hepatic lymphangiomatosis mimicking polycystic liver disease

Hepatic lymphangiomatosis is a rare disorder characterized by cystic dilata tion of the lymphatic vessels in the hepatic parenchyma, It can occur in th e liver alone, in the liver and spleen, or in multiple organs. Clinically, diagnosis can be difficult because of the rarity and protean manifestations of this disorder. We describe a 53-year-old woman with hepatic lymphangiom atosis in whom polycystic liver disease had been previously diagnosed. In a ddition, we review 12 cases of hepatic, splenic, and hepatosplenic lymphang iomatosis with or without systemic lymphangiomatosis and discuss the differ ential diagnosis.