Navarra G, Occhionorelli S, Mascoli F, Santini M, Benea G, Marzola A
Institute of General Surgery, University of Ferrara, Italy.
J Cardiovasc Surg (Torino). 1994 Aug;35(4):333-6.
Leiomyosarcoma of vascular origin is an extremely rare lesion especially when it occurs in the arteries. In the present study, we report a case of such a neoplasm, originating in the wall of the descending aorta and give an ample review of world references on the subjects of primary aortic neoplasm. Wright classified aortic tumors into two categories concerning the site of origin in the aortic wall: the first involves the intima, the second group consists of tumors arising in the media or adventitia. The aspecific clinical findings that characterize these lesions explain the difficulty of a preoperative diagnosis. Very important, therefore, are sonography and CT which point out signs and symptoms which refer to a local and distal diffusion of the tumor. However, even in the cases in which the diagnosis is done before surgery, there is no codified therapeutic management. In fact both surgical and non-surgical methods (radiotherapy, chemotherapy) have poor results with an average survival. Due to the limited prognosis "quoad vitam", the elective therapy must preferably be of conservative type.
血管源性平滑肌肉瘤是一种极其罕见的病变，尤其是发生在动脉时。在本研究中，我们报告了一例起源于降主动脉壁的此类肿瘤病例，并对原发性主动脉肿瘤相关的世界文献进行了详尽综述。赖特根据主动脉壁的起源部位将主动脉肿瘤分为两类：第一类涉及内膜，第二类包括起源于中膜或外膜的肿瘤。这些病变的非特异性临床表现解释了术前诊断的困难。因此，超声检查和CT非常重要，它们能指出提示肿瘤局部和远处扩散的体征和症状。然而，即使在术前做出诊断的病例中，也没有规范化的治疗方案。事实上，手术和非手术方法（放疗、化疗）的效果都很差，平均生存期较短。鉴于“生命”预后有限，选择性治疗最好采用保守型。
