Primary malignant tumors of the aorta: clinical presentation, treatment, and course of different entities

Böhner, H.; Luther, B; Braunstein, Stefan; Beer, Sandra; Sandmann, W.

doi:10.1016/s0741-5214(03)00935-2

Cited by 55 publications

(26 citation statements)

References 14 publications

(26 reference statements)

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“…Ultrasound, CT, magnetic resonance imaging, and magnetic resonance angiography have been used to diagnose aortic sarcomas [1,[6][7][8][9]; however, the correct diagnosis has rarely been made. To our knowledge, this is the second report to document the use of conventional aortography for diagnosing an intimal sarcoma of the aorta before autopsy.…”

Section: Fig 1 Transverse (Top Left) and Sagittal (Top Right) Ct Scmentioning

confidence: 99%

Primary sarcoma of an abdominal aortic aneurysm

et al. 2005

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Primary tumors of the aorta are extremely rare and the diagnosis is made most often after surgery or autopsy. Because clinical symptoms of abdominal sarcoma are similar to those of occlusive or aneurysmal disease, aortic sarcomas are frequently mistaken for these lesions. The imaging findings are frequently nonspecific and therefore do not allow a definitive preoperative diagnosis. We report a case of an epithelioid angiosarcoma in the vessel wall of an abdominal aortic aneurysm.Keywords : abdominal ortic aneurysm ; Sarcoma ; Aortography ; Diagnosis Primary tumors of the aorta are extremely rare and almost always malignant. Most aortic tumors are often confused for occlusive or aneurysmal atherosclerotic lesions and are diagnosed mainly after surgery or autopsy. We report a case of an epithelioid angiosarcoma in the vessel wall of an abdominal aortic aneurysm (AAA). Case reportA 50-year-old man was referred to our department with a known AAA that was 46 mm in diameter and a wall thrombus diagnosed by abdominal computed tomography (CT; Fig. 1A,B). The patient complained of left intermittent claudication, lumbar pain, sexual impotence, and significant weight loss (25 kg) in the preceding year. These physical problems were associated with psychological disorders. Aortography confirmed the diagnosis of a small aneurysm with subocclusion of the left common iliac artery (Fig. 1C). An irregular plaque of the infrarenal aorta was also observed. Aortic surgery was done to alleviate the occlusive lesions of the iliac artery and the small AAA. Aortotomy revealed an unusual cystic wall thrombus at the level of the terminal aorta and left common iliac artery. A sample of the thrombus including the aortic wall was excised for histologic analysis. An aortobiiliac bifurcated graft was inserted.The histologic investigation showed irregular, polymorphic large cells grouped in poorly cohesive sheets (Fig. 2). Atypical mitotic figures were observed. These cells showed positive immunohistologic staining for vimentin, CD31, CD34, and factor VIII but were negative for epithelial markers and smooth muscle actin. This pattern was suggestive of an epithelioid angiosarcoma.Postoperatively, a whole-body positron emission tomography, scintigraphy and CT were performed to search for a primary lesion and potential metastasis. Intense fluorine 18 fluorodeoxyglucose ( 18 F-FDG) uptake was observed at the level of the terminal aorta and at the level of right and left femurs, iliac artery, and vertebral body (Fig. 3). CT and scintigraphy demonstrated several lytic lesions in the bones of the lower part of the body (vertebral body L4, right and left iliac bones, sacrum, neck of the right femur, patella, and condyle of the left iliac bone). These osteoclastic lesions and some erosions were suggestive of metastases.

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Section: Fig 1 Transverse (Top Left) and Sagittal (Top Right) Ct Scmentioning

confidence: 99%

Primary sarcoma of an abdominal aortic aneurysm

et al. 2005

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“…Therefore, the patients' general symptoms, such as weight loss and fever, were the only symptom of inflammatory malignant disease, whereas signs of vasculitis may appear in cases of epitheloid angiosarcoma [7]. Due to the aggressive nature of the tumor, prognosis is poor, and there is high rate of metastatic disease at the time of diagnosis; the most common site of mestastasis is the bone.…”

Section: Discussionmentioning

confidence: 99%

“…Most cases of epitheloid angiosarcoma reported in the literature have described an association with the foreign material Dacron in surgically implanted grafts [7,9]. However, in the only case published so far, Umscheid et al [10] reported a high-grade epithelioid angiosarcoma arising at the abdominal aortic wall after implantation of a Vanguard endograft.…”

Section: Discussionmentioning

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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“…Other case reports of intimal angiosarcoma also noted an ESR Ͼ100 mm/h, and one noted an ESR of 70 mm/h. 7,8 It has been suggested that one should suspect an aortic sarcoma if a patient presents with symptoms of arterial occlusion or thromboembolism, a normal echocardiogram result, and the finding of a heterogeneous protruding aortic plaque in the absence of generalized atherosclerotic disease. 3,8,9 In concert with these findings, an elevated ESR, particularly if it is Ͼ100 mm/h, may increase one's suspicion for the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Postoperative outcomes in intimal aortic angiosarcoma: A case report and review of the literature

Nicotera

Simosa²,

Campbell³

2009

Journal of Vascular Surgery

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Primary malignant tumors of the aorta: clinical presentation, treatment, and course of different entities

Abstract: Different types of malignant aortic tumors seem to have different clinical presentation and course.

Cited by 55 publications

References 14 publications

Primary sarcoma of an abdominal aortic aneurysm

Primary sarcoma of an abdominal aortic aneurysm

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

Postoperative outcomes in intimal aortic angiosarcoma: A case report and review of the literature

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