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Experience with hepatic hemangioendothelioma in infancy and childhood
G W Holcomb1, J A O'Neill, S Mahboubi
1Department of General Surgery, Children's Hospital of Philadelphia, PA 19104.
Insights
Hepatic hemangioendothelioma, a liver tumor in infants, is often diagnosed clinically but confirmed with imaging. Steroids are a primary treatment, with embolization or ligation for non-responders, achieving an 80% survival rate.
Area of Science:
- Pediatric Oncology
- Hepatology
- Diagnostic Imaging
Background:
- Hepatic hemangioendothelioma is a rare liver tumor affecting infants and children.
- Common presentations include hepatomegaly, congestive heart failure, and cutaneous lesions.
Purpose of the Study:
- To report on the experience with 16 pediatric patients diagnosed with hepatic hemangioendothelioma.
- To evaluate diagnostic modalities and treatment outcomes for this condition.
Main Methods:
- Retrospective review of 16 pediatric cases with hepatic hemangioendothelioma.
- Analysis of diagnostic methods including arteriography and computed tomography (CT).
- Assessment of various treatment strategies: radiation, resection, steroids, hepatic artery ligation, and embolization.
Main Results:
- Computed tomography (CT) with enhancement demonstrated diagnostic specificity comparable to hepatic arteriography.
- Steroids proved effective for most patients; embolization or ligation were successful for non-responders.
- Hemangioendotheliomatosis with dual arterial supply (portal and hepatic) did not respond to embolization.
- Overall survival rate in the series was 80%.
Conclusions:
- CT is a reliable diagnostic tool for hepatic hemangioendothelioma.
- Systemic steroids are a primary treatment modality.
- Embolization is effective only when the tumor is supplied by the hepatic artery alone.
Abstract:
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%.