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Aortic angiosarcoma is a rare, malignant neoplasm of the vasculature, with 24 case reports in the literature. Patients usually present with either aneurysmal or occlusive disease. Treatment consists of en bloc resection of the vessel and contiguous structures with postoperative chemoradiation therapy. Despite surgery and adjunctive measures, prognosis remains dismal as a result of early metastasis and late diagnosis. We report a case of aortic angiosarcoma that presented with distal thromboembolic phenomenon.

The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. Survival at 5 years was 8%. Survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection.

Aortic aneurysms and stenosis are widely treated with endovascular procedures in the current era. In this report, we present endovascular stent grafting for symptomatic treatment of severe thoracic aortic stenosis caused by an aortic sarcomatoid carcinoma in a 73-year-old female. The peri- and post-operative courses were complicated with tumor embolisms that were managed by both endovascular and surgical measures.

Primary angiosarcoma of the aorta is a rare malignancy that is characterized by rapid proliferation and propensity for metastasis. It has been reported only 35 times in the surgical literature. This case report presents a 66-year-old man diagnosed with angiosarcoma of his native aorta 7 years after endograft repair of an abdominal aortic aneurysm. We then reviewed the world surgical literature for occurrence, tumorigenic studies, prognosis, and management of aortic angiosarcoma. Because native aortic tissue is retained after endovascular repair of an abdominal aortic aneurysm, the treating physician should have an awareness of this pathology and entertain the diagnosis as appropriate.