HAEMANGIOENDOTHELIOMA SCANNING

Haemangioendothelioma
Vascular tumours account for most benign liver tumours in childhood, with haemangioendotheliomas being seen more frequently than cavernous haemangiomas. Although haemangioendothelioma may be asymptomatic, infants generally present before the age of 6 months with an abdominal mass, respiratory distress, anaemia and cardiac failure, caused by the shunting of blood from the aorta through the tumour.

Large tumours may bleed spontaneously, resulting in haemoperitoneum. They may present with jaundice and increased transaminase levels and 50% of children also have cutaneous haemangioma. These tumours are generally multiple, of varying echogenicity and may have a complex echotexture due to thrombus, calcifications and internal septations.

The vascular nature of these lesions is demonstrated by a large coeliac axis and marked decrease in the size of the aorta below the origin of the coeliac axis. The main differential diagnosis of multiple haemangioendothelioma is from metastatic liver disease, particularly from disseminated neuroblastoma.

Although most asymptomatic paediatric haemangioendotheliomas regress spontaneously, those complicated by cardiac failure require active treatment. Steroids may be administered and serial ultrasound scans may be used to monitor the gradual resolution of the lesion.

Angiographic embolization or surgical ligation of the major feeding vessels of the hepatic artery may be necessary in severe cases that fail to respond to steroid therapy.