Management of a rare case of intimal sarcoma of the aortic arch is reported, which was diagnosed unexpectedly after total arch replacement for pseudoaneurysm. The prognosis for this condition is poor, with death usually within a few months from diagnosis. The newly developed proton-beam radiation therapy was applied to treat a local recurrence of the sarcoma following surgery. Positron-emission tomography/computed tomography revealed complete remission of the lesion.
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