Intimal angiosarcoma of the thoracic aorta

Sarcomas of the great vessels are uncommon, with aortic being the rarest. Only 30 cases of true intimal aortic sarcomas (IAS) are documented. They tend to occur in the abdominal aorta, with less common occurrences in the thoracic aorta. Their growth patterns, predispose them to a propensity for metastases and cause embolic phenomenon. A 58-year-old male presented with chest pain and dyspnea and was evaluated for pulmonary embolus and coronary artery disease. Computed tomography angiography (CTA) demonstrated no pulmonary emboli; however, there was severe atherosclerosis/thrombosis of the aortic arch. The process extended centrally, nearly filling the entire lumen. The surgery consultant advised anticoagulation and strict blood pressure control, recommending that the patient come to the outpatient department for surgery. Due to personal reasons, the patient failed to return at the recommended time. Three months after initial presentation the patient was admitted for surgical replacement of the aorta. The surgeon reported the aorta as chock-full of fibro-fatty material nearly obstructing its course. The pathology report was aortic sarcoma of intimal origin. Aortic sarcomas are rare tumors, with the intimal subtype in the thoracic aorta being even rarer. Delay in diagnosis of these tumors often occurs, since the imaging features are nearly identical to atherosclerotic disease. Since atherosclerotic disease is clearly more frequent than intimal sarcoma of the aorta, it is not difficult to understand that this diagnosis is not usually made until after surgical resection or at autopsy. At presentation, nearly all of the patients have metastatic disease.