Liver involvement in Langerhans' cell histiocytosis: a study of nine cases.

Nine cases of Langerhans' cell histiocytosis (LCH) of the liver are presented. Five of the patients had liver involvement only. Other organ systems, notably the lymph nodes and skin, were involved in the other four patients. Four of the patients had sclerosing biliary disease with infiltration of the bile ducts by Langerhans' cells, whereas in two other patients, the biliary sclerosis was not associated with direct hepatic involvement by Langerhans' cells. Histologically, the lesions were composed of focal aggregates of Langerhans' cells in a polymorphous background of mature eosinophils, lymphocytes, neutrophils, and plasma cells. LCH encompasses a syndrome that has a broad range of clinical presentations and that might involve the liver solely as tumor-like lesions or cystic lesions, or as part of systemic disease. Even when Langerhans' cells are not demonstrable, sclerosing cholangitis can be seen in LCH.