Background: Epithelioid hemangio-endothelioma (EHE) is a rare malignant tumor of vascular origin that electively metastasizes to lungs and liver. Case report: We report the case of a 51-year-old woman with metastatic and progressive EHE of the liver. She was successfully treated with daily administration of sunitinib malate, an oral multi-target tyrosine kinase inhibitor, which particularly targets vascular endothelial growth factor (VEGF) receptors. Stabilization has lasted 6 years. We discuss the rationale for using antiangiogenic treatment for EHE. Conclusion: This case adds to a growing body of evidence that suggests that antiangiogenic agents should be considered in patients who present with advanced EHE.

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