Experience with hepatic hemangioendothelioma in infancy and childhood.

This report describes our experience with 16 infants and children with a mean age of 7 months and a median age of 6 weeks with hepatic hemangioendothelioma. Fifteen patients presented with hepatomegaly. Seven had congestive heart failure and four had associated cutaneous lesions. Although diagnosis was clinically evident in 15 of the 16 patients, arteriography and computerized tomography (CT) were diagnostic. A variety of treatment approaches were used including radiation, resection, systemic steroids, hepatic artery ligation, angiographic embolization, and various combinations of these modalities. The following information was gained from this experience. CT with enhancement is as specific a diagnostic tool as hepatic arteriography. Angiographic and CT appearances do not correlate with prognosis. Most patients can be treated successfully with steroids; those who do not respond should have other approaches tried such as embolization or ligation. The angiographic appearance determines whether embolization therapy is worthwhile, as we found that hemangioendotheliomatosis with portal as well as hepatic arterial supply will not respond to embolization. The survival rate in this series was 80%.