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Hepatic hemangioendothelioma: clinical experience and management strategy
J A Daller1, J Bueno, J Gutierrez
1Thomas E. Starzl Transplant Institute, University of Pittsburgh and Children's Hospital of Pittsburgh Transplantation Surgery, PA 15213, USA.
Insights
Resectional surgery, with or without liver transplantation, offers better survival rates for infantile hepatic hemangioendothelioma than embolization. Medical management failure necessitates surgical intervention for improved outcomes.
Area of Science:
- Hepatology
- Pediatric Surgery
- Vascular Anomalies
Background:
- Infantile hepatic hemangioendothelioma (IHH) is a rare benign liver tumor in infants.
- Management strategies for IHH vary, impacting patient outcomes.
Purpose of the Study:
- To define optimal management strategies for infantile hepatic hemangioendothelioma based on clinical experience.
- To identify factors predicting mortality in infants with IHH.
Main Methods:
- Retrospective analysis of 13 patients with IHH treated between 1989 and 1997.
- Evaluation of diagnostic modalities including ultrasonography and CT scans.
- Comparison of outcomes for different treatment strategies: medical, surgical resection, embolization, and liver transplantation.
Main Results:
- Congestive heart failure and abdominal mass were predictive of 5-month mortality.
- Resectional surgery, with or without liver transplantation, resulted in significantly lower 5-month mortality and higher 2-year survival rates compared to hepatic artery ligation or embolization.
- Late morbidity and mortality were associated with the chosen treatment modality.
Conclusions:
- Resectional therapy is recommended for IHH when medical management fails.
- Hepatic artery embolization can serve as a definitive or temporizing measure before liver transplantation if resection is not feasible.
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.