Primary sarcoma of an abdominal aortic aneurysm

Abstract

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.

Primary angiosarcoma of the abdominal aorta: multi-row computed tomography

“…Embolic occlusion of peripheral or mesenteric arteries occurs as well as intermittent claudication, abdominal pain, back pain, or metastatic complications [4,[6][7][8]. Aortic angiosarcoma may even coincide with pathology and clinical symptoms of abdominal aortic aneurysm [7].…”

“…Nevertheless, since most patients with these tumors are older than 60 years, most cases will be coincidental with generalized atherosclerotic, or even aneurysmal vascular changes and will become apparent through metastasis or will be diagnosed postoperatively [6,7].…”

“…In conclusion, intimal angiosarcoma is rare, but should be included in the differential diagnosis in case of polypoid mural alterations without aneurysm and corresponding atherosclerotic changes in other parts of the vascular system [7,8].…”

“…It arises from intimal endothelial cells (intima type) and myofibroblastic cells (mural type). Only a few cases have been reported so far [1][2][3][4][5][6][7][8].…”

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

“…Fewer than 140 cases of aortic sarcoma have been described in the literature, and about 40 of those were classified as intimal sarcoma [1][2][3][4][5][6] . These tumours are found mostly in the abdominal aorta, and their clinical presentation depends on the precise site of occurrence 2,3,[7][8][9] . A definitive diagnosis is often made only after surgery or even autopsy.…”