Primary Tumors of the Thoracoabdominal Aorta: Surgical Treatment of 5 Patients and Review of the Literature

Chiche, Laurent; Mongrédien, Benoît; Brochériou, Isabelle; Kieffer, Édouard

doi:10.1007/s10016-003-0018-x

Cited by 93 publications

(109 citation statements)

References 47 publications

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“…Fewer than 140 aortic sarcomas have been reported in the literature, with only 10 defined as angiosarcoma of the infrarenal aorta [1][2][3]. The median survival rate of patients with aortic sarcomas has been reported to be 7 months, which was explained by a late diagnosis combined with inadequate surgical resection [1].…”

Section: Discussionmentioning

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: Discussionmentioning

confidence: 99%

Primary sarcoma of an abdominal aortic aneurysm

et al. 2005

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“…Five year survival is reported at 12% after surgical intervention, and only 8% after conservative treatment. 294 Endovascular repair is not recommended. Stent grafts do not preclude the growth and metastatic potential of In pa ents with Marfan syndrome, trans-thoracic echocardiographic measurement of the aor c root should be considered for the diagnosis and follow up of aor c involvement IIa C 274…”

Section: Recommenda On 64mentioning

confidence: 99%

Editor's Choice – Management of Descending Thoracic Aorta Diseases

Riambau¹,

Böckler²,

Brunkwall³

et al. 2017

European Journal of Vascular and Endovascular Surgery

915

259

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“…The diagnosis of vascular leiomyosarcoma is often missed because the clinical manifestations are non-specific and vary widely according to the location of the tumor, and because no reliable tumor marker is available. Unlike leiomyosarcoma of the inferior vena cava (2-4), aortic leiomyosarcoma is an extremely rare and very ominous tumor (5,6). Herein, we present a case of primary leiomyosarcoma of the abdominal aorta presenting with renovascular hypertension due to tumor embolization in the renal arteries.…”

Section: Leiomyosarcoma Of the Abdominal Aorta: A Rare Cause Of Renovmentioning

confidence: 96%

“…Aortic tumors are classified into two categories according to the site of occurrence in the aortic wall: the intimal-type tumors and the mural-type tumors (6,9). This case belongs to the former group.…”

Section: Fig 3 Clinical Coursementioning

confidence: 99%