Hepatic hemangioendothelioma. Angiographic appearance and apparent prednisone responsiveness
C. Jackson, H. L. Greene, J. O'Neill and S. Kirchner
A 6-month-old girl was seen because of multiple cutaneous hemangiomas,
hepatomegaly, and failure to thrive. A liver scan with technetium Tc 99m
sulfur colloid showed two filling defects in the left lobe. Diffuse nodular
hepatic lesions characteristic of hemangioendotheliomas were disclosed by
celiac angiography and confirmed by biopsy. Congestive heart failure,
present in many infants with this syndrome, was not manifest. Prednisone
was administered, 2 mg/kg, on alternate days. Liver size and cutaneous
hamangiomas promptly regressed in size and "catch up" linear growth
occurred during six months of treatment. Congestive heart failure did not
develop. Sixteen months after treatment, liver size and a second hepatic
scan were normal.