rimary malignant lesions of the aorta occur rarely and are associated with a very low survival rate. Common presentations are distal embolic phenomena, 1 aortic dissection, 2 or even occlusion of the aortic lumen. 3 This case report describes a patient with a primary malignant fibrous histiocytoma (MFH) of the thoracic aorta associated with ulcer-like projection (ULP). The aortic mural tumor invaded into the surrounding tissue, not into the aortic lumen, and the radiological appearance mimicked ULP with aortic pseudoaneurysm formation. To our knowledge, a primary aortic tumor with ULP has not been previously reported and knowledge of the characteristic imaging findings is clinically important.
Case ReportA 57-year-old woman with a 1-month history of heartburn and who suffered from progressive back pain was admitted to hospital. Her past medical history was rheumatoid arthritis. Physical examination revealed: blood pressure, 130/ 90 mmHg; pulse, 96 beats/min; respiration, 16 breaths/min; and body temperature, 36.8°C. Lung and cardiac sounds were normal. Blood count revealed slight anemia (hemoglobin level: 10.5 g/dl). Other laboratory data, including blood chemistry, coagulation studies, and cardiac enzymes, were not abnormal. Precontrast and contrast-enhanced dynamic computed tomographic (CT) imaging was performed using a 64-row multislice scanner (Aquilion-64, Toshiba). Contrast agent (100 ml) at an iodine concentration of 300 mg/ml (Iomeron, Eisai) was injected at a rate of 4 ml/s, and arterial phase imaging was performed 30 s later. The delay time was 3 min for delayed phase imaging. CT images in the arterial phase showed a 6.5×4.3×6.7 cm aortic Circulation Journal Vol.71, October 2007 lesion with ULP in the thoracic descending aorta (Fig 1).The attenuation values (in Hounsfield units (HU)) were measured at regions of interest (ROIs) in the aortic lesion in each phase. Attenuation of each ROI was 53 HU, 59 HU, and 62 HU on the precontrast CT, arterial-phase, and delayed-phase CT images, respectively. Because the case was initially diagnosed as a pseudoaneurysm with ULP and possible impending rupture, emergency endovascular stentgraft placement was selected as the treatment, using a tailored 100-mm stent-graft device (Gianturco Z-stent with UBE woven Dacron graft material). The immediate post interventional result showed complete exclusion and thrombosis of the ULP (Fig 2). One month after the stent -graft treatment, the ULP was completely excluded but the aortic mass lesion showed no regression in size on CT imaging. Two months later, the aortic mass lesion had increased in size and there was distinct enhancement on contrastenhanced CT imaging (Fig 3). The patient was referred for surgery during which it was found that a tumor of the thoracic aorta had solidly invaded the pulmonary hilus and was considered to be nonresectable. Surgical biopsy was performed. On histological examination, the tumor comprised a haphazardly arranged mixture of pleomorphic spindle and giant cells with frequent mitosis. Immunohis...