PRIMARY LEIOMYOSARCOMA OF THE AORTA - REPORT OF A CASE AND REVIEW OF THE LITERATURE

Leiomyosarcoma of vascular origin is an extremely rare lesion especial ly when it occurs in the arteries. In the present study, we report a c ase of such a neoplasm, originating in the wall of the descending aort a and give an ample review of world references on the subjects of prim ary aortic neoplasm. Wright classified aortic tumors into two categori es concerning the site of origin in the aortic wall: the first involve s the intima, the second group consists of tumors arising in the media or adventitia. The aspecific clinical findings that characterize thes e lesions explain the difficulty of a preoperative diagnosis. Very imp ortant, therefore, are sonography and CT which point out signs and sym ptoms which refer to a local and distal diffusion of the tumor. Howeve r, even in the cases in which the diagnosis is done before surgery, th ere is no codified therapeutic management. Infact both surgical and no n-surgical methods (radiotherapy, chemotherapy) have poor results with an average survival. Due to the limited prognosis ''quoad vitam'', th e elective therapy must preferably be of conservative type.