Primary aortic intimal sarcoma of the endothelial cell type with long-term survival

Abstract

Primary tumors of the aorta are rare and are difficult to diagnose preoperatively. These tumors are malignant and ultimately fatal. A patient had initial evidence of aortoiliac obstructive disease. Aortic endarterectomy produced an excellent postoperative clinical result until the pathology report from the endarterectomy specimen revealed aortic sarcoma. The patient refused a second operation for en bloc aortic resection. One year later with recurrent symptoms, the patient underwent en bloc resection of the aorta and common iliac vessels until clear margins were obtained. The tumor was found to be intimal aortic sarcoma of endothelial cell origin. The only metastasis was to bone, and it was controlled with radiation and chemotherapy. The patient lived a total of 8 years from the time of initial diagnosis. Abdominal aortic intimal sarcoma of the endothelial cell type is a rare variety of aortic sarcoma that can be managed successfully if diagnosed early. This specific type of tumor has a longer survival period and better prognosis than other types of aortic sarcoma when managed with aggressive surgical resection, radiation, and chemotherapy.