J Pediatr Surg 1999 Jan;34(1):98-105; discussion 105-6

Hepatic hemangioendothelioma: clinical experience and management strategy

Daller JA, Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS, Mazariegos
G, Reyes J

Abstract
PURPOSE: This study sought to define management strategies based on clinical
experience in treating infantile hepatic hemangioendothelioma. METHODS: A
retrospective analysis of patients with hemangioendothelioma presenting to a
tertiary liver transplantation center between 1989 and 1997 was performed.
RESULTS: Thirteen patients (median age, 14 days) with hemangioendothelioma were
identified. Congestive heart failure (P<.03) and abdominal mass (P<.081) were
predictive of 5-month mortality rates. Ultrasonography and computerized axial
tomography were the diagnostic modalities most commonly used. Treatment
strategies consisted of medical management (steroids and alpha-interferon) and
interventional modalities (hepatic artery ligation or embolization, resectional
surgery, or orthotopic liver transplantation). Patients who underwent
resectional surgery, with or without orthotopic liver transplantation, had a
lower 5-month mortality rate (P<.02) and a greater 2-year survival rate (P<.003)
than did those who underwent hepatic artery ligation or embolization. Early
morbidity and mortality tended to be a consequence of the primary lesion,
whereas late morbidity and mortality were reflective of the treatment modality
used. CONCLUSIONS: In cases of failed medical management, resectional therapy
should be used when possible. If partial hepatectomy is not technically
achievable, hepatic artery embolization should be used either as definitive
therapy or as a temporizing measure until orthotopic liver transplantation is
possible