Infantile Hemangioendothelioma
Grace E. Kim, MD
Key Facts
Clinical Issues
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Most common hepatic neoplasm in 1st year of life
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> 90% present before age of 6 months
Microscopic Pathology
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Vascular proliferation resembling small capillaries
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Slightly dilated and irregular in shape
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Lined by single layer of bland endothelial cells
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Top Differential Diagnoses
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Angiosarcoma
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Previously termed infantile hemangioendothelioma type 2
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Key distinguishing feature is nuclear atypia of endothelial cells
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This partial hepatectomy specimen contains a poorly circumscribed, white-tan mass  with central calcification. |
A proliferation of small, thin-walled vascular channels is present within a fibrous stroma containing entrapped bile ducts  . |
TERMINOLOGY
Synonyms
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Originally described by Dehner and Ishak as infantile hemangioendothelioma type 1
Definitions
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Hepatic vascular neoplasm
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Most common hepatic neoplasm during 1st year of life
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Accounts for 15% of primary liver tumors in pediatric population
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CLINICAL ISSUES
Epidemiology
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Age
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Most present before age of 6 months
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Rare in adults
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