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Infantile hemangioendothelioma of the liver
D M Selby1, J T Stocker, M A Waclawiw
1Office of the Deputy Director, Armed Forces Institute of Pathology, Washington, D.C. 20306.
Insights
Infantile hemangioendothelioma of the liver is a rare tumor affecting infants, often presenting within the first six months. Early detection of congestive heart failure and multiple nodules improves survival prediction.
Area of Science:
- Pediatric Oncology
- Hepatology
- Pathology
Background:
- Infantile hemangioendothelioma (IHE) of the liver is a rare vascular tumor in infants.
- Early diagnosis and understanding of prognostic factors are crucial for management.
Purpose of the Study:
- To report clinical and morphological findings in a cohort of 91 patients with liver IHE.
- To identify factors predicting mortality in infants with liver IHE.
Main Methods:
- Retrospective review of clinical data and pathological findings from 91 patients diagnosed with liver IHE.
- Analysis of immunohistochemical and electron microscopy findings.
- Statistical analysis to determine predictors of 6-month survival.
Main Results:
- Most patients (87%) were diagnosed before 6 months of age.
- Common clinical features included anemia, hyperbilirubinemia, and elevated AST. Congestive heart failure occurred in 15% of patients.
- The 6-month survival rate was 70%. Significant predictors of mortality included congestive heart failure, jaundice, and multiple tumor nodules.
Conclusions:
- Liver IHE is a significant cause of morbidity and mortality in infants.
- Clinical presentation and tumor characteristics like multiple nodules and absence of cavernous differentiation are key prognostic indicators.
- Advanced diagnostic methods did not significantly improve practical assessment compared to clinical and morphological features.
Abstract:
Clinical and morphological findings in 91 patients with infantile hemangioendothelioma of the liver are reported. The study comprised 56 girls and 35 boys ranging in age from premature infant to 3 yr; one outlier patient was 18 yr old. Most patients with infantile hemangioendothelioma (87%) were first seen before the age of 6 mo. Congestive heart failure was evident in 15%. Skin hemangiomas were noted in 11%. Anemia, hyperbilirubinemia and increased AST level were present. Solitary lesions were more common than multiple ones (3:2). Immunohistochemical staining of tumor cells for factor VIII was positive in 20 of 21 cases tested; testing for blood group antigen was positive in 8 of 28 cases. Cytokeratin staining verified the presence of bile ducts, some of which appeared to be the result of transformation of injured liver. No pericytes were identified on electron microscopy. The 6-mo survival rate, based on 71 cases, was 70%. Average time of follow-up for the survivors was 7.7 yr. All deaths occurred during the initial presentation/hospitalization of infants, with the exception of two patients who died 3 mo and 7 mo after diagnosis. More recent analytic methods, including immunohistochemical stains and flow cytometric studies, do not contribute to the practical assessment of this tumor. Covariates with significant value in predicting death 6 mo after diagnosis included presence of congestive heart failure, jaundice, multiple tumor nodules and absence of cavernous differentiation.