Embolizing intimal sarcoma - a rare cause of peripheral arterial occlusion

E. Arn, D. Becker, P. Stalder, C. Binkert, P. Wigger

Objective

Aortic intimal sarcoma is an aggressive and rare type of vascular tumor. Most reported cases present with embolisation and involve the descending thoracic and abdominal aorta.

Methods

A 83-year-old man presented with acute ischemia of the right lower extremity. He was anticoagulated because of atrial fibrillation with an INR of 2.9 on admission. The Angio-CT-scan of the pelvis and lower extremity showed an embolic occlusion of the right tibiofibular trunk and bilateral aneurysm of the common iliac artery. Aspiration embolectomy was successfully performed. Because of the atypical appearance of the removed embolus, a histopathologic examination was performed, which showed a suspicion of myxoid neoplasia. A transthoracal echocardiography showed no signs of an atrial myxoma. Two month later he presented with a repeat embolic occlusion of the right leg. A thoraco-abdominal CT scan showed an endoluminal mass in the thoracic aorta, which we interpreted as the embolizing myxoma. In consideration of age and comorbidities of the patient we decided to cover the tumor with a stentgraft to prevent further embolization. Unfortunately the patient developed postoperatively a Brown-Sequard-Syndrom and an ileum ischemia due to intraoperative embolization. The revised histopathologic report of the embolic material in the intestinal arteries showed an intimal sarcoma.

Results

The most common cause of peripheral arterial occlusion is cardioembolic. Aortic aneurysm or progressive arteriosclerosis can be other sources of peripheral arterial embolization. The intraaortic sarcoma is rather rarely reported in the literature but overall the most common type of primary malignant tumors of the aorta. The symptoms are due to tumor embolisation with ischemia of extremities or specific organs. Tumor suspicious emboli should undergo histopathologic evaluation. The histological interpretation is difficult, so immunohistochemical stain is demanded to differentiate from other endoluminal tumors.

Conclusion

Uncommon sources for peripheral arterial embolization should be considered if suspicious thrombotic material is found. In these cases an histopathologic work-up of the thrombotic material can be helpful to identify intraarterial tumors as an embolic source.

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