Abstract
Primary aortic sarcoma is a rare condition that is frequently associated with distal embolization. In addition, growth characteristics of primary aortic sarcoma lead to the narrowing of the involved aortic lumen. A 72-year-old Korean male with primary aortic sarcoma showed progressive unexplained blood pressure elevation that didn’t improve with additional antihypertensive drug therapy. Because follow-up measures were not taken, the patient ultimately developed hypertensive encephalopathy with concurrent embolic dissemination. Although we successfully performed open transcatheter embolectomy in both legs, the patient died because of multiple organ failure 3 days after surgery. Given the ominous prognosis for this condition, this case report highlights the fact that the value of early detection and prompt evaluation of altered vital signs should not be overemphasized. We describe a rare case of primary aortic sarcoma that showed hypertensive encephalopathy caused by thoracic aortic occlusion and also had embolic metastases to the lower extremities.
Highlights
Primary aortic sarcoma (PAS) is a very rare and aggressive malignancy that has been frequently associated with embolic events associated with broken pieces of tumor
We present a rare case of PAS that required open transcatheter embolectomy for the treatment of malperfusion due to metastatic emboli to the lower extremities, with concurrent hypertensive encephalopathy caused by thoracic aortic occlusion
The intimal type of aortic sarcoma forms protruding intraluminal masses that can lead to embolic dissemination through the bloodstream [1]
Summary
Primary aortic sarcoma (PAS) is a very rare and aggressive malignancy that has been frequently associated with embolic events associated with broken pieces of tumor. If aortic luminal narrowing becomes significant, hypertensive encephalopathy may occur because of the backward pressure overload, as with coarctation of the aorta In this case report, we present a rare case of PAS that required open transcatheter embolectomy for the treatment of malperfusion due to metastatic emboli to the lower extremities, with concurrent hypertensive encephalopathy caused by thoracic aortic occlusion. Total occlusion of the right common iliac artery and left superficial femoral artery were noted in 3DCT scans of the lower extremities (Figure 1B). Both renal arteries were maintaining patency without any filling defect. This histopathologic report confirmed the diagnosis of intimal-type PAS
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