Isolated hepatic hemangiomatosis in adults: Report of two cases and review of the literature

Hepatic hemangiomatosis is a rare condition characterized by diffuse replacement of liver parenchyma with tissue similar to that of cavernous hemangioma. The purpose of this report is to illustrate two adult cases of hepatic hemangiomatosis and to review the pathological, radiological and clinical issues of this condition. The first case is that of a 47-year-old woman with extensive involvement of the entire liver. The right lobe was almost totally replaced, and only a limited amount of residual hepatic parenchyma was present in the left lobe. The second case is that of a 31-year-old woman with less extensive involvement. The right lobe was almost totally involved, and was accompanied with a large hematoma. The left lobe was relatively unaffected. Both cases were complicated by consumption coagulopathy (Kasabach-Merritt syndrome). The patients were successfully managed surgically, with living donor liver transplantation in the first case and extensive right lobectomy in the second case. The condition described by the term hepatic hemangiomatosis includes a wide clinical spectrum, with some cases asymptomatic, some accompanied with shunting, and some resulting in rupture or life-threatening hepatic failure. The disease has a good overall prognosis, with surgery and arterial embolization as effective treatment options.