Hemangioendothelioma of the hepatobiliary system: the classic and the unusual.

Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.