Intimal Sarcoma of the Aorta

Mohsen, Nancy; Haber, Marian M.; Urrutia, Victor C.; Nunes, Linda White

doi:10.2214/ajr.175.5.1751289

Cited by 55 publications

(26 citation statements)

References 3 publications

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“…Radiology plays an important role in their diagnosis, but it can be difficult to differentiate between an aortic hypovascular tumor and atherosclerotic disease, both in clinical manifestation and radiological appearance. 1,5,12 In the present case, the aortic tumor involved the mural layer, which showed intra-and extra-mural growth patterns, and no significant filling defect within the aortic lumen. An irregular aortic surface localized to the tumor and the circumferential extent of the tumor were seen on CT imaging (Fig 1), but the aortic tumor showed only little enhancement on dynamic CT imaging, and thus the differentiation between ULP with aneurysmal formation and aortic sarcoma was difficult.…”

Section: Discussionmentioning

confidence: 45%

“…14 Contrast-enhanced MRI, however, is possibly useful in differentiating tumor from atherosclerotic disease because of the higher contrast resolution. 1 Also, we believe positron emission tomography may be helpful for diagnosis of the aortic tumor.…”

Section: Discussionmentioning

confidence: 94%

“…Fewer than 100 aortic sarcomas have been reported in the world literature. 1,[5][6][7][8][9] They occur throughout the aorta and are most common in middle-aged patients, with mean age 54 years. 6 There is a male-to-female ratio of 2:1, with an average survival of 1.5 years after diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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

Utsunomiya

Ikeda

Ideta

et al. 2007

Circ J

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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...

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Section: Discussionmentioning

confidence: 45%

Section: Discussionmentioning

confidence: 94%

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

Utsunomiya

Ikeda

Ideta

et al. 2007

Circ J

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“…Most patients present with clinical symptoms attributable to embolic phenomena or vessel occlusion or obstruction 2,3,[7][8][9]12,13 . Rarely, presentations such as cerebral infarction, deep-vein thrombosis, gastrointestinal hemorrhage, and ulcerated ischemic bowel have been reported 10,14,15 .…”

Section: Discussionmentioning

confidence: 99%

Uncommon Case of Brain Metastasis in a Patient with a History of Heavy Smoking

2014

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Primary sarcomas of the aorta are extremely uncommon. Depending on histomorphology and immunohistochemical pattern, intimal sarcomas can show angiosarcomatous differentiation. Here, we describe the case of a 60-year-old woman with a primary intimal sarcoma of the aortic arch and signs of cerebral metastatic disease as the initial manifestation. After the patient experienced the onset of severe headaches, ataxia, and left-sided weakness, magnetic resonance imaging showed several brain lesions. Histologic assessment of a brain biopsy specimen revealed a malignant tumour composed of large pleomorphic cells that were positive for pancytokeratin and CD10. Radiation to the brain did not significantly improve the patient’s symptoms, and cranial computed tomography (ct) imaging revealed several metastases, indicating lack of response. Because of the patient’s smoking history, the presence of central nervous system and skeletal metastases on combined positron-emission tomography and ct imaging, and the focal pan-cytokeratin positivity of the tumour, carcinoma of the lung was favoured as the primary tumour. Despite chemotherapy with cisplatin and etoposide, the patient’s neurologic symptoms and general condition deteriorated rapidly, and she died within a few days. At autopsy, an undifferentiated intimal sarcoma of the aortic arch was diagnosed. The primary tumour in the aorta consisted of large pleomorphic cells. Immunohistochemical analysis of the aortic tumour and brain metastases demonstrated diffuse positivity for vimentin and p53 and focal S-100 staining. In summary, we report a challenging case of advanced intimal sarcoma of the aortic arch with brain and bone metastases at initial presentation. Our report demonstrates the difficulties in diagnosing and treating this disease, and the need for multicentre studies to accrue more patients for investigations of optimal therapy.

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“…Primary malignancies of the aorta represent a rare but aggressive tumor entity and constitute approximately 1-2% of soft-tissue sarcomas [1]. As a subtype of angiosarcoma, epithelioid angiosarcomas have a low incidence and only rarely arise from the larger blood vessels themselves [2].…”

Section: Introductionmentioning

confidence: 99%

Epithelioid Angiosarcoma With Metastatic Disease After Endovascular Therapy of Abdominal Aortic Aneurysm

Schmehl

Scharpf

Brechtel

et al. 2011

Cardiovasc Intervent Radiol

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Malignancies of the aortic wall represent a rare condition, and only a few reports have covered cases of sarcomas arising at the site of a prosthesis made of Dacron. A coincidence with endovascular repair has only been reported in one case to date. We report a patient with epithelioid angiosarcoma and metastatic disease, which was found in an aneurysmal sac after endovascular aortic repair for abdominal aortic aneurysm.

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Intimal Sarcoma of the Aorta

Cited by 55 publications

References 3 publications

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

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

Uncommon Case of Brain Metastasis in a Patient with a History of Heavy Smoking

Epithelioid Angiosarcoma With Metastatic Disease After Endovascular Therapy of Abdominal Aortic Aneurysm

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