Abstract
Hepatic hemangioendotheliomas are rare childhood tumors. Infantile hepatic hemangioendothelioma (IHHE) is the most common vascular tumor of the liver in children accounting for 12% of all childhood hepatic tumors. We report an 8-month-old female infant presented at the age of 4 months with incidental hepatomegaly. On presentation her abdomen was soft and lax. The liver was at 8 cm below the right costal margin, not tender with rounded edge and nodular surface. The spleen was not felt and there were no other masses and no ascites. Her liver functions tests were normal. MRI of the liver showed numerous rounded lesions in all the hepatic segments with characteristic of low signal intensity at T1 and high intensity at T2. Post contrast, the lesions showed a peripheral thick rim of enhancement and in the delayed porto-venous phase showed central filling with contrast. The portal and hepatic veins were patent. Our findings were compatible with the diagnosis of multifocal infantile hemangioendothelioma of the liver. Oral prednisone was started for 2 weeks then was tapered over 4 months. A follow up MRI confirmed the decrease in the number and the size of hepatic lesions. A follow-up liver US 24 months after treatment showed residual solitary small lesion in left hepatic lobe measuring 1.2 x 1.0 cm. The authors report that steroid therapy was effective in the treatment of massive multifocal HUE without a need for other advanced therapies including liver transplantation.