Malignant Fibrous Histiocytoma Arising From the Aortic Wall Mimicking a Pseudoaneurysm With Ulceration

Abstract

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...

High-Grade Undifferentiated Pleomorphic Sarcoma of the Aortic Arch: A Case of Endovascular Therapy for Embolic Prophylaxis and Review of the Literature

“…Several reports of endovascular interventions in patients with PMTAs are found within the literature. [23][24][25] However, in each case, the diagnosis of malignancy had not yet been established and the presenting symptoms were thought to represent primary aneurismal or athero-embolic disease. Although the use of endovascular intervention for embolic prophylaxis in unresectable tumors has been suggested by other investigators, 2,26 to our knowledge this case represents the first endovascular surgery for a previously diagnosed PMTA.…”

2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease: Executive Summary

“…Symptoms may include malaise, fatigue, weight loss and nausea or the occurrence of distal arterial embolization (with histopathologic examination showing neoplasm, or identified by imaging techniques during a search for an embolic source). [216][217][218] AoD may originate in the area of the neoplasm or the aortic occlusion. 219 Resection and reconstruction of the segment of aorta containing the neoplasm have been described, but because most patients present with metastatic disease, overall prognosis is poor.…”

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta Presenting with Ruptured Saccular Aneurysm: A Case Report

“…등 통증, 신고혈압 등을 일으킬 수 있으며 원위부 색전 현상이나 대동맥 폐색의 증상으로도 발현될 수 있다( 2 ). 대동맥 벽에서 기원하여 궤양성 동맥류를 형성하는 경우는 극히 드물게 증례 보고가 되어 있으며 대부분 수술 전에 악성 종양으로 인지되지 못하였다( 3 4 ). 본 증례도 관통성죽상경화 궤양성 동맥류나 진균성 동맥류(mycotic aneurysm) 파열로 의심되어 혈관 내 흉부 대동맥 치료(thoracic endovascular aortic repair; 이하 TEVAR)를 시행하여 동맥류는 소실되었으나 6개월 추적 흉부 전산화단층촬영(이하 CT)에서 스텐트 그라프트 주위에 종괴가 발생하여 시행한 조직검사에서 미분화성 다형성 육종으로 진단되었다.…”

“…PET 소견 상 종양의 과대사성 활동(hypermetabolic activity)으로 인해 FDG 섭취가 비후된 대동맥 벽에서 증가하였으며 흉추에도 전이성 병변이 발견되었다. 따라서, CT에서 대동맥에 국소적으로 소낭성 동맥류가 있고 대동맥 벽의 편심성 비후 및 조영증강이 있으면서 다른 대동맥은 정상인 경우, 일차성 대동맥 육종이 원인 질환일 가능성을 반드시 고려하여야 하며 MRI와 PET 등이 대동맥 육종을 진단하는데 도움이 될 수 있다( 4 8 10 )…”