3 month old male child came with clinically enlarged nodular palpable liver. CT scan shows enlarged liver with multiple peripherally enhancing variable sized nodular lesion seen occupying whole of the liver in both the lobes. Infra renal part of aorta is small in caliber (arrow head) with dilated and prominant celiac axis (not shown here). There is also similar lesion seen in the upper back subcutaneous region on right side. Features suggestive of hemangioendothelioma.
Benign hemangioendothelioma is a rare liver neoplasm, however it represents the most common vascular tumor of the liver in the neonate.
The neonatal clinical presentation of hemangioendothelioma includes:
1) gross liver enlargement,
2) high-output cardiac failure, and
3) associated hemangiomas in other organs including skin (hemangiomatosis).
Other complications include consumptive coagulopathy, hemolytic anemia, tumor rupture, and problematic surgical intervention. Hemangioendotheliomas have been recognized on obstetric ultrasound. Neonatal CT, repeat sonography, MRI imaging, and arteriography have all been utilized to visualize the tumor and assess it's size, location, and the anatomy of arterio-venous malformation (AVM) in the neonate.
The antenatal ultrasound features of hemangioendothelioma include: liver mass (heterogeneous, hypoechoic, complex with anechoic spaces, and hyperechoic), consequent liver enlargement, prominent vasculature, cardiomegaly, and possible hydrops (serous effusions and body edema).Color and pulsed Doppler interrogation can also be particularly useful in identifying an AVM.Post natal findings are liver enlargement, multiple or single confluent liver masses showing peripheral enhancement, may find AV malformation. Typically the celiac axis will be dilated and reduced caliber of infra renal aorta. May find extrahepatic hemangioendothelioma.