Primary aortic intimal sarcoma

Aortic intimal angiosarcoma is extremely rare, and the prognosis of patients with a tumor is unfavorable even if they have undergone surgery. We treated a patient with primary intimal angiosarcoma of the aortic arch who underwent an operation. The tumor originated from the inner wall of the aortic arch on the lesser curve. In order to remove the tumor completely, the entire aortic arch from the ascending to the middle of descending aorta was resected through an L-shape skin incision. On histologic examination, an undifferentiated intimal sarcoma was diagnosed. It grew into the aortic lumen while spreading along the aortic intima, focally infiltrating the media. The postoperative course was uneventful. Postoperative CT performed at 6, 12 and 18 months after surgery showed no local recurrence or metastasis. According to some reports endoarterectomy has been performed to treat this type of tumor, since the malignant cells are thought to be limited to the luminal surface. However, we favor aortic resections and graft interpositions rather than an endoarterectomy because the tumor could have invaded the media.