Epithelioid Sarcoma-Like Hemangioendothelioma
Steven D. Billings, MD
Key Facts
Terminology
Vascular tumor of apparent intermediate malignancy that histologically closely resembles epithelioid sarcoma
Clinical Issues
Presents most commonly in young adults
Microscopic Pathology
Ill-defined nodules, sheets, or short fascicles
Predominantly epithelioid morphology
Immunophenotype: CD31(+), cytokeratin(+), FLI-1(+), vimentin(+), CD34(-)
No multicellular vascular channels present
Only rare intracytoplasmic lumen formation in approximately 1/2 of cases
Top Differential Diagnoses
Epithelioid sarcoma
Epithelioid hemangioendothelioma
 Hematoxylin & eosin demonstrates ill-defined nodular growth pattern of epithelioid sarcoma-like hemangioendothelioma. |
 Hematoxylin & eosin shows epithelioid tumor cells with relatively abundant eosinophilic cytoplasm. The tumor transitions from epithelioid areas to more spindled areas in the left side of the image. |
TERMINOLOGY
Definitions
Vascular tumor of apparent intermediate malignancy that histologically closely resembles epithelioid sarcoma
CLINICAL ISSUES
Epidemiology
Age
Most common in young adults (range: 17-70 years)
Site
Most common in lower extremity
Presentation
May present in superficial or deep soft tissue
Cutaneous lesions may be ulcerated
Treatment
Surgical approaches
