[Hepatoportal sclerosis and liver angiosarcoma: an infrequent association with a possible common etiology]

Hepatoportal sclerosis is characterized by fibrosis of the intima of the portal vein and its branches leading to the development of presinusoidal portal hypertension. We describe the case of a 58-year-old woman with idiopathic hepatoportal sclerosis, who was admitted to our service due to impairment of liver function, with rapid clinical worsening and finally the development of multiorgan failure. Autopsy showed a diffuse liver angiosarcoma with splenic metastases. The patient had no history of domestic or occupational exposure to substances involved in the development of hepatoportal sclerosis or liver angiosarcoma. The development of liver angiosarcoma in a patient with hepatoportal sclerosis is exceptional, even though both diseases may have a common etiology.