Introduction: Epithelioid hemangioendothelioma (EHE) is a rare endothelial tumor with an intermediate grade of malignancy. Few cases of primary vascular hemangioendothelioma have been described in the literature. Surgery is the treatment of choice, but radiation therapy and chemotherapy should also be considered in particular cases.
Case presentation: We present the case of a 44-year-old Caucasian woman affected by EHE of the cephalic vein, treated by complete surgical removal of the mass and postoperative local radiation therapy. At 5-year follow-up, our patient is alive, with no signs of local or distant relapse and with no late radiation-related effects.
Conclusions: Postoperative radiotherapy may play a role in cases in which tumor margins are close or cannot be assessed or when high-risk features are present.