Eur J Cardiothorac Surg 2002;21:574-576
© 2002 Elsevier Science NL
Intimal-type primary sarcoma of the thoracic aorta: an unusual case presenting with left arm embolization
Giulio Pompilioa*,
Paolo Tartaraa,
Chiara Varesib,
Paolo Bigliolia
a Department of Cardiovascular Surgery, University of Milan, Centro Cardiologico Monzino Foundation IRCCS, Via Parea 4, 20138 Milano, Italy
b Institute of Pathological Anathomy, University of Milan, Milano, Italy
Received 18 October 2001;
received in revised form 31 December 2001;
accepted 31 December 2001.
* Corresponding author. Tel.: +39-02-58002562; fax: +39-02-58011194
e-mail: giulio.pompilio{at}cardiologicomonzino.it
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Abstract
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A case of intimal-type primary sarcoma of the thoracic aorta with unusual left arm embolization is reported.
Key Words: Aortic sarcoma • Peripheral embolization • Magnetic resonance imaging
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1. Introduction
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Intimal-type sarcomas of the thoracic aorta are rare primary tumors, characterized by an aggressive behavior [1]. No more than 90 cases are described in the literature [2]. The clinical presentation is often non-specific and make diagnosis sometimes extremely difficult.
We here report a rare case of an undifferentiated sarcoma of the descending thoracic aorta presenting with unusual embolization of the left arm.
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2. Case report
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A 39-year-old man was initially admitted to another hospital with an acute ischemia of the left arm. At angiography occlusion of the third distal segment of the left homeral artery was shown, treated subsequently with surgical embolectomy. Histological examination of the thromboembolic mass showed an undifferentiated sarcoma of high-grade malignancy. The patient's postoperative work-up was directed toward identifying a source for the tumor emboli. Chest film and echocardiography were negative. An elevated CA 19.9 level (65 U/ml) was the only abnormal blood value. Computed tomography (CT) of the chest and abdomen showed a lytic lesion into the left second rib, and a vegetating mass in the thoracic cavity arising from it. Contrast CT-scan of the chest also revealed slightly non-vegetating filling defects of the descending thoracic aorta, considered as intraluminal atherothrombotic plaques. No intra-cardiac abnormalities were observed. The embolic mass found in the homeral artery was considered as a metastasis causing an acute thrombosis. Then, a surgical resection both of the second rib and of the neoplastic mass were performed and four cycles of chemotherapy with Adriamycine and Iphosphamide were started.
The patient remained well and asymptomatic. Thirteen months later, a control magnetic resonance imaging (MRI) and a thoracic aortography revealed an intraluminal aortic mass, extending for about 5 cm of length in the descending thoracic aorta near the origin of the left subclavian artery (Fig. 1
). These findings were considered to consist of a sarcoma of the descending thoracic aorta. The presence of a little pulmonary metastasis in the left inferior lobe was also revealed by CT-scan.
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Fig. 1. Aortography (A) and MRI (B), showing an intraluminal filling defect in the proximal descending thoracic aorta (arrows). Notice that MRI allows to distinguish tumor from surrounding normal tissue.
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The patient was therefore admitted to our institution for surgical treatment and scheduled for an urgent operation. The chest was entered through a posterolateral incision in the bed of the fourth rib. The aortic arch and the descending thoracic aorta were carefully isolated, in an attempt to avoid embolic phenomena from the tumoral mass. The proximal and middle descending thoracic aorta and the origin of the left subclavian artery were resected. An extensive tract of the descending aorta was replaced with a Dacron tube according to the ‘quick simple clamping technique’ previously described [3]. The subclavian artery was directly reimplanted on the vascular graft. A resection of the pulmonary metastasis and of the periaortic tissue, including some lymphatic nodes and the origin of four couples of intercostal branches was also performed. The postoperative period was uneventful until the 9th day, when persistent abdominal pain occurred. An abdominal CT-scan showed a large splenic infarct that required an explorative laparotomy. Splenectomy was accomplished, with an eventful postoperative outcome.
Two months later, a new tumoral mass in the left thigh was found, treated with palliative radiotherapy. The clinical conditions of the patient remained stable for 5 months and chemotherapy was continued. Afterwards, because of worsening of the clinical status and back pain, the patient was readmitted to the hospital. A skeletal scintiscan and a CT-scan of the chest revealed multiple bone (ribs and vertebral bodies) metastases. The patient died of cachexia, 22 months after the initial embolic event, and 9 months after descending thoracic aorta resection.
2.1. Histopathologic features
The specimen of the resected aortic wall was histologically examined, and an undifferentiated intimal sarcoma was diagnosed, according to Burke and Virmani classification [1]. Pathologic findings of the resected specimen included a vegetating and ulcerated neoplastic mass measuring 4.8x3 cm2, invading the full thickness of the aortic wall and the periadventitial tissue. On histologic examination, a high undifferentiated, pleomorphic sarcoma was observed (Fig. 2A
), showing positivity for vimentin and smooth muscle actin, and negativity for desmin, S-100 protein, lysozime, FVIII and cytokeratine (Fig. 2B). Proliferating cell nuclear antigen (PCNA) reaction showed high neoplastic cell proliferation. Ultrastructural examination confirmed the diagnosis of a tumor of myofibroblastic origin [1,4]. The gross appearance of the aorta is shown in Fig. 2C.
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Fig. 2. (A) Histology of the intimal sarcoma showing pleomorphic spindle-cell pattern with numerous abnormal mitotic figures. (Hematoxylin–Eosin stain; x400). (B) Staining for Vimentine is strongly positive in this sarcoma, which has a myofibroblastic origin. Notice also the storiform growth, beside necrosis areas and ghost cells. (Avidin–Biotin Complex, x250). (C) Gross appearance of the distal part of the resected thoracic aorta, showing a polypoid and ulcerated tumoral mass.
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3. Discussion
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This report is meant to highlight the difficulties of an early diagnosis of primary aortic sarcomas, and the poor specificity in their clinical presentation.
Non-specific symptoms (weight loss, fever, anorexia) and embolic phenomena are the most frequent clinical presentation of aortic sarcomas [1]. Embolization generally occurs in intimal-type tumors [4]. At the moment of diagnosis, metastases have been described in over 50% of patients, their sites being liver, spleen, kidneys, adrenal glands, mesentery, small bowel, oesophagus, pancreas, skin, lungs, pleurae and bones.
Tumoral thromboembolic phenomena in the peripheral arteries leading to ischemia have been observed in the legs or in visceral arteries [4]. To our knowledge, this is the first report of left arm embolization as clinical presentation. This may have contributed to a delayed diagnosis after the initial embolic event. As suggested by Higgins [4], a primary aortic tumor should be suspected in case of evidence of tumoral emboli once a cardiac source is excluded. Thus, the histologic examination of any peripheral embolism seems mandatory. Another possible confounding factor was the low accuracy in detecting aortic tumors by means of a CT-scan only. As previously suggested [5–7], both CT-scan and aortography are unable to distinguish tumors from surrounding normal tissue or intraluminal atherosclerotic thrombi. Higgins et al. [4] have advocated MRI superiority in detecting arterial wall sarcomas.
Although never described before, another useful tool for differential diagnosis may be considered for the elevation of the blood level of the tumoral marker CA 19.9, found abnormal in this case from the moment of the initial embolic event. This finding needs further confirmation, but maybe, should be considered if an aortic tumor is suspected.
A mean survival of 8–14 months is expected when metastases are found [2,7]. Survival after aortic resection is often fear [2,7,8], as the case we are reporting (9 months). However, the delay between clinical onset and aortic resection was in this patient more than 1 year. It is hoped that improvements in early diagnosis could ameliorate postoperative survival, even in case of undifferentiated very aggressive aortic sarcomas.
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Acknowledgments
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We thank Mrs Graziella Alfonsi and Mrs Marina Crippa for their excellent technical assistance.
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References
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Abstract
1. Introduction
