Infantile Hepatic Hemangioendothelioma is a rare benign vascular tumour of liver , usually presenting in infancy with cardiac failure.

Benign hepatic tumors account for about one third of all hepatic tumors in children. The majority of these tumors are of vascular origin, usually hemangioendotheliomas or cavernous hemangiomas. Infantile Hepatic Hemangio Endothelioma ( IHHE) is a type of capillary hemangioma, which is seen almost exclusively in children.

Presentation: It is the most common symptomatic vascular tumor, with 85% of cases presenting in the first 6 months of life. The tumor may appear as an asymptomatic abdominal mass or more commonly present with congestive heart failure, thrombocytopenia, jaundice or hemorrhage. The heart failure is usually secondary to shunting within the tumour. Additionally , 50% of patients have cutaneous haemangiomas

On Ultrasonography, the liver showed gross hepatomegaly involving both the lobes with multiple mixed echoic lesions of varying sizes, ranging from 2 to 4 cm.

On Doppler study, the blood vessels supplying these masses were tortuous and dilated, and demonstrated nonphasic low resistance brisk blood flow. The Celiac axis showed marked increase in flow velocity. Parasagittal view of abdominal aorta showed enlarged aortic caliber proximal to the celiac axis, with marked tapering of the aorta distal to the celiac axis. The hepatic veins also showed very high flow velocities suggestive of arterio-venous shunting.

On CT abdomen , liver showed massive enlargement and was studded with multiple markedly enhancing nodules of varying sizes involving both the lobes suggestive of haemangioendotheliomas.

Three other liver tumors should be considered as differentials: hepatoblastoma, mesenchymal hamartoma, and metastatic neuroblastoma. Hepatoblastoma is rarely seen in children less than one year of age. The alpha-fetoprotein level is usually markedly elevated, whereas in cases of infantile hemangioendothelioma, it is usually normal or only mildly to moderately increased. By CT, hepatoblastoma is more heterogenous than infantile hemangioendothelioma, especially after contrast administration. Mesenchymal hamartomas usually occur in the infant and are not associated with elevated alpha- fetoprotein levels. Mesenchymal hamartoma typically appears as multilocular cystic masses, rarely as a solid lesion. When solid, mesenchymal hamartomas appear as avascular or hypo vascular masses on angiography, scintigraphy, and Doppler ultrasound. Metastatic neuroblastoma may be confused with the multicentric form of infantile hemangioendothelioma. With metastatic neuroblastoma, urinary levels of catecholamines are almost always elevated, while the primary tumor, often an adrenal mass, can be seen. On contrast enhanced CT scans, marked enhancement is usually noted in cases of infantile hemangioendothelioma, while with metastatic neuroblastoma enhancing areas present normal residual liver.

The child died after 1 week due to congestive heart failure. Autopsy specimen showed the liver studded with multiple nodules in both the lobes.