Epithelioid Vascular Tumors: Understanding the Differences

• Brief overview of epithelioid vascular tumors.
• Importance of distinguishing between different subtypes for diagnosis and treatment.

1. Epithelioid Hemangioma
   • Clinical Features: Benign nature, slow growth.
   • Morphology: Well-defined nodules of epithelioid endothelial cells.
   • IHC: Positive for CD31, CD34, ERG and FOS B.
   • Molecular Findings: FOS rearrangement in a third of epithelioid hemangioma across different locations and histologic variants.
   • Prognosis: Excellent prognosis, rarely metastasizes.
2. Epithelioid Hemangioendothelioma
   • Clinical Features: Intermediate behavior, locally aggressive.
   • Morphology: Sheets or cords of epithelioid endothelial cells, infiltrative growth.
   • IHC: Positive for CD31, ERG, CAMTA1.
   • Molecular Findings: WWTR1-CAMTA1 fusion in > 90 % cases and YAP-TFE3 
   • Prognosis: Variable prognosis, metastasis possible.
3. Epithelioid Angiosarcoma
   • Clinical Features: Malignant, aggressive behavior.
   • Morphology: Pleomorphic, epithelioid cells with high mitotic activity.
   • IHC: Positive for CD31, CD34, ERG.
   • Molecular Findings: TP53 mutations, other genetic abnormalities.
   • Prognosis: Poor prognosis, high risk of metastasis.

Some important quick revision cards

References: WHO Tumors of the bone and soft tissue 2020 - 5th edition.

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