A "High-Risk" Epithelioid Hemangioendothelioma Presenting as a Solitary, Ulcerated, Subcutaneous Tumor

So-called "high-risk" epithelioid hemangioendotheliomas are uncommon neoplasms that demonstrate classic histopathologic features of epithelioid hemangioendotheliomas and a size larger than 3 cm or >3 mitotic figures per 50 high power fields. These neoplasms show an increased rate of metastasis (25% of cases) and are associated with a poor 5-year survival (59%). They may pose a diagnostic challenge for dermatopathologists as they mimic metastatic carcinoma, malignant mixed tumor, melanocytic neoplasms, epithelioid sarcoma, and epithelioid angiosarcoma. High-risk epithelioid hemangioendothelioma has not been previously reported as a solitary ulcerated mass in the skin. Here, we describe one such lesion that developed as a rapidly growing ulcerative skin tumor in a 33-year-old African American man with a remote history of Burkitt lymphoma. We also review the evolution and controversies in the understanding and classification of this neoplasm.