Epithelioid hemangioendothelioma is a rare endothelial cell-derived tumor of intermediate grade malignancy. It has an unpredictable outcome, independently of the usual histoprognostic criteria. Thirty-three cases with intravascular localisation are described in the literature. We describe a case occurring in a 31-year-old male, which developed an inferior veina cava tumor, with recurrence after incomplete surgical resection and chemotherapy. He was operated on again under extracorporeal circulation and complete surgical resection was then performed. Histologic examination found an intravascular epithelioid hemangioendothelioma developing in the vascular lumen without vascular wall infiltration. This entity represents a challenge not only for the vascular surgeon, but also for the pathologist given its difficult diagnosis.
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