INFANTILE HEMANGIOENDOTHELIOMA OF THE LIVER REVISITED

Infantile hemangioendothelioma is a benign tumor of the liver composed of anastomosing vascular channels lined by plump endothelial cells. A t initial presentation, most patients are 6 months of age or younger a nd have hepatomegaly or an abdominal mass. Congestive heart failure, b leeding, anemia, jaundice, and cutaneous or visceral hemangiomas may a lso be present. Grossly, the lesions are usually well circumscribed an d may be focal, multifocal, or diffuse. Large solitary lesions are oft en associated with central hemorrhage or necrosis. Radiography reveals a mass that is occasionally calcified. Angiography reveals hypervascu lar lesions, often with arteriovenous shunting. A solid lesion with va riable echotexture is noted at ultrasound. Computed tomography typical ly shows a low-attenuation solid lesion with peripheral enhancement. C entral enhancement is often lacking except in smaller lesions. At magn etic resonance imaging performed with T2-weighted pulse sequences, the lesions usually have high signal intensity. Spontaneous regression of the tumor occurs, although patients may die of associated conditions.