Malignant fibrous histiocytoma arising from descending thoracic aorta

Cases of malignant fibrous histiocytoma arising from the aorta are rare and have a dismal outlook despite treatment. The longest reported survival period following resection is only 28 months. A patient with malignant fibrous histiocytoma of the descending thoracic aorta was successfully treated with resection and reconstruction with a prosthetic graft. The patient developed metastatic disease eight months postoperatively. With aggressive triple regimen chemotherapy, complete remission has been obtained. The patient is alive and free of disease six years postoperatively.