Hemangioendothelioma

• Rare tumor
• Intermediate-grade malignant tumors, clinically behaving between benign hemangioma and malignant angiosarcoma. Some have rather benign course while others show much more malignant progression
• Typically occur in adults with roughly equal sex distribution
• Most common location is soft tissues, but have been described essentially anywhere in the body
• Symptoms depend upon location of the primary tumor
• Most common histologic type is epithelioid hmangioendothelioma (EHE)
• For isolated tumors, curative resection with negative margin should be performed
• Role of adjuvant RT is unclear, but is often considered
• Most common metastatic sites are lungs, liver, lymph nodes and bone
• Effectiveness of chemotherapy is unclear
• Most studies are small single institution series