Infantile Hemangioendothelioma
Grace E. Kim, MD
Key Facts
Clinical Issues
Most common hepatic neoplasm in 1st year of life
> 90% present before age of 6 months
Microscopic Pathology
Vascular proliferation resembling small capillaries
Slightly dilated and irregular in shape
Lined by single layer of bland endothelial cells
Top Differential Diagnoses
Angiosarcoma
Previously termed infantile hemangioendothelioma type 2
Key distinguishing feature is nuclear atypia of endothelial cells
 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
Originally described by Dehner and Ishak as infantile hemangioendothelioma type 1
Definitions
Hepatic vascular neoplasm
Most common hepatic neoplasm during 1st year of life
Accounts for 15% of primary liver tumors in pediatric population
CLINICAL ISSUES
Epidemiology
Age
Most present before age of 6 months
Rare in adults
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