Epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma 

Updated: 02/28/2023

© Jun Wang, MD, PhD

General features
  • Intermediate grade
  • Commonly associated with a vein
  • Usually adults, more common in women
  • High mortality rate if in lung
Clinical presentations
  • Reddish-brown lobulated masses
  • Hemorrhagic appearance
  • More common in extremities, head and neck, mediastinum, trunk
Key morphological features
Markers
  • Positive for endothelial markers factor VIII–related antigen, CD31, and CD34
  • Positive for lymphatic marker D2-40
  • Positive for smooth muscle marker: smooth muscle actin
  • Positive for CAMTA1
Molecular abnormality
  • WWTR1-CAMTA1 gene rearrangement
Treatment
  • Low grade: wide excision
  • High grade: radical excision with lymph node dissection


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Contents

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