Epithelioid hemangioendothelioma of the azygos vein

The authors report a case of epithelioid hemangioendothelioma of the azygos vein diagnosed during the investigation of a superior vena cava syndrome. Venography and thoracic CT and nuclear magnetic resonance scans showed an endovenous tumour with spinal and lymph node invasion. Cavographic-controlled biopsies allowed histological identification of the tumour. Palliative therapy consisted in tumour ablation and superior vena cava bypass. Epithelioid hemangioendothelioma is a rare vascular tumour characterised by the presence of epithelioid endothelial cells. It is slowly progressive and its potential malignancy justifies surgical excision whenever possible.