Epidemiology

 more common in younger women than in men

Associations (mostly when lesions are multiple)

 hepatic adenoma
 hepatic hemangioma (23%)
 vascular anomalies in the brain berry aneurysms
 Local ischemia

• sickle cell anemia

 patent ductus venosus
 intrahepatic shunts
 Congenital absence of the portal vein
 Hepatic vein thrombosis
 Cystic dysplasia of the kidneys
 Klippel-Trenauny syndrome
 Inflammatory pseudotumor of the liver
 meningioma, astrocytoma
 arterial dysplasia
 HHT hereditary hemorrhagic telangiectasia
 glycogen storage diseases
 adjacent to

• hydatid cyst
• hemangioma
• fibrolamellar carcinoma
• epithelioid hemangioendothelioma

 ingestion of oral contraceptives

Pathological synopsis

 single nodule, anywhere within the liver parenchyma, usually clearly demarcated from the rest of the liver, but lacking a well defined capsule
 macroscopical central scar (50%)
 multiple pseudolobules with fibrovascular and ductular areas radiating from perilobular septa (80%)
 fibrous bands
 thick-walled vessels
 reactive ductules
 +/- teleangiectatic features
 +/- hyperplastic and even adenomatous parts
 +/- large-cell dysplasia has been described

Complications

 Hepatic vein obstruction
 Peripheral fatty infiltration
 Kasabach-Merrit syndrome

Differentail diagnosis

 cirrhosis
 hepatocellular adenoma