[Aortic sarcoma. A rare pathology]

In the light of a case of endotheliosarcoma of the thoracic aorta, the authors found in the literature 33 cases of aortic sarcomas and 13 of sarcomas of peripheral arteries. The clinical diagnosis of this rare tumor is difficult. The diagnosis can be made before surgery only on the basis of angiographic and CT scan findings, whilst in most instances common arterial lesions are suspected whether of atheromatous or embolic origin. The positive diagnosis of arterial sarcoma remains histological. The diagnosis of an endothelial tumor can be made with the help of the techniques of factor VIII, antigen H and, more recently, lectins. Surgical treatment by excision and restoration of continuity has most often been used. Alone or in combination with chemo and/or radiotherapy, it would seem to be inadequate to prevent progression which involves the invasion of adjacent tissues, tumor emboli or generalized metastases. The prognosis of such a tumor remains gloomy with an average life expectancy of one year and 7 months, regardless of the histological type of arterial sarcoma.