Mesenteric infarction secondary to tumor emboli from primary aortic sarcoma. Guidelines for diagnosis and management

Higgins, Robert; Posner, Mitchell C.; Moosa, Hans H.; Staley, Charles A.; Pataki, Kenneth I.; Mendelow, Harvey

doi:10.1002/1097-0142(19911001)68:7<1622::aid-cncr2820680726>3.0.co;2-b

Cited by 82 publications

(52 citation statements)

References 5 publications

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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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“…The choice of treatment for undifferentiated pleomorphic sarcoma is local surgical resection (3,(5)(6)(7). The small number of cases assessed so far does not allow ultimate evaluation of the role of adjuvant chemotherapy and radiotherapy (4).…”

Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, no fewer than 24 cases of undifferentiated pleomorphic sarcoma originating from the aorta have been reported in the worldwide literature, but imaging findings have been rarely reported (3)(4)(5)(6)(7).…”

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confidence: 99%

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta: A Case Report

Heo

Park

Kim

et al. 2016

J Korean Soc Radiol

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Primary sarcomas of the great vessels are extremely uncommon tumors, with aortic sarcoma being the rarest type. They tend to occur in the thoracic aorta, followed by the abdominal aorta and the thoracoabdominal aorta. They have a propensity for metastases and they cause acute arterial embolism (1).Undifferentiated pleomorphic sarcoma, also called malignant fibrous histiocytoma, has been defined as a group of pleomorphic, high-grade sarcomas that fail to show any line of differentiation using currently available ancillary techniques (2). To the best of our knowledge, no fewer than 24 cases of undifferentiated pleomorphic sarcoma originating from the aorta have been reported in the worldwide literature, but imaging findings have been rarely reported (3-7).Herein, we describe the case of a 67-year-old male who had a primary undifferentiated pleomorphic sarcoma of the descending thoracic aorta with an emphasis on computed tomography (CT) and magnetic resonance imaging (MRI) findings. CASE REPORTA 67-year-old Russian male presented with a 5-month history of epigastric and back pain. The patient had a history of diabetes mellitus and hypertension. Laboratory studies revealed abnormal liver function test (gamma-glutamyl transpeptidase:143 IU/L, alkaline phosphatase: 448 IU/L) and increased C-reactive protein (1.98 mg/dL). Electrocardiogram showed normal sinus rhythm. Contrast enhanced abdomen and chest CT (64-slice multidetector CT, GE Healthcare, LightSpeed VCT, Milwaukee, WI, USA) were performed. Abdomen CT showed no significant findings, and contrast-enhanced CT of the patient's chest showed a heterogeneously enhancing lobulated mass encasing the right aspect of the descending thoracic aorta, extending from the T6 to T8 level, and measuring 1.8 × 3.5 × 3.2 cm in size (Fig. 1A, B). The mass occupied 20% of the aortic lumen, and the intimal layer of the aorta was intact. Thoracic Undifferentiated pleomorphic sarcoma, known as malignant fibrous histiocytoma, arising from the wall of the aorta is an extremely rare tumor. We report computed tomography and magnetic resonance imaging findings of a 67-year-old male patient with histopathologically proven undifferentiated pleomorphic sarcoma arising from the wall of the descending thoracic aorta. To the best of our knowledge, this is the first report of such a case in Korea. Knowledge of imaging findings of undifferentiated pleomorphic sarcoma will be helpful for accurate diagnosis and appropriate management.

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“…Clinical presentation depends on the tumour site. 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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Mesenteric infarction secondary to tumor emboli from primary aortic sarcoma. Guidelines for diagnosis and management

Cited by 82 publications

References 5 publications

Primary sarcoma of an abdominal aortic aneurysm

Primary sarcoma of an abdominal aortic aneurysm

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta: A Case Report

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

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