Asian Cardiovasc Thorac Ann 2003;11:352-354
© 2003 Asia Publishing EXchange Ltd
Repair of Ascending Aortic Aneurysm in a Patient with Fibrosarcoma over Sternum
Enver Dayioglu, MD,
Yusuf Kalko, MD,
Murat Basaran, MD,
Ertan Onursal, MD
Department of Cardiovascular Surgery, Istanbul University, Çapa, Turkey
For reprint information contact: Murat Basaran, MD Tel: 90 212 534 0000 Fax: 90 212 534 2232 email: dr_murat_basaran{at}hotmail.com Istanbul University, Department of Cardiovascular Surgery, Millet Caddesi, Çapa, Istanbul 34390, Turkey.
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ABSTRACT
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The combination of an ascending aortic aneurysm and suprasternal soft tissue tumor is rarely reported in the literature. This case involves a 62-year-old man diagnosed with an ascending aortic aneurysm and a fibrosarcoma over the sternum that was successfully treated by surgery. The patient underwent an aortic replacement operation with a Dacron graft and a muscle flap transposition procedure to cover the defect over the sternum.
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INTRODUCTION
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Ascending aortic aneurysms are among the most common potentially fatal aortic diseases. The pathology requires urgent surgical intervention to prevent aortic rupture or dissection. This report involves a 62-year-old man diagnosed with ascending aortic aneurysm and a fibrosarcoma over the sternum that was successfully treated by surgery.
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CASE REPORT
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A 62-year-old man diagnosed with a fibrosarcoma was referred to our clinic with increasing shortness of breath and chest pain. He was hemodynamically stable with a blood pressure of 130/65 mm Hg. On admission, pulse and respiratory rates were 92 and 23 per minute respectively, and there was a grade 1/6 systolic murmur on auscultation. All peripheral pulses were palpable and symmetrical. Laboratory data were within the normal limits, except for an elevated erythrocyte sedimentation rate of 58 mm•h-1. Transthoracic echocardiography showed an ascending aortic aneurysm (AAA) with a diameter of 5.2 cm. The aortic valve annulus was normal in size and there was no aortic insufficiency. Further diagnostic evaluation with computed tomography confirmed the initial diagnosis revealing an ascending aortic aneurysm and a tumor over the sternum (Figure 1). Collaborating with the Plastic and Reconstructive Surgery Department, an elective concomitant operation was undertaken for both the ascending aortic aneurysm and suprasternal fibrosarcoma.
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Figure 1. Computed tomography revealing an ascending aortic aneurysm and a large fibrosarcoma over sternum.
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The median sternotomy was performed after radical excision of the large subcutaneous tumor over the sternum. The pericardium was loosely attached to the aneurysmal sac and there was non-hemorrhagic pericardial effusion. The sac measured 5.2 cm and was filled with a layered thrombus. The sinotubular ridge was preserved and the aortic annulus was not dilated. The aortic diameter just beyond the origin of the innominate artery was normal. The patient was placed on cardiopulmonary bypass with distal ascending aortic and two-stage cannulations. A vent was inserted into the left ventricle through the right superior pulmonary vein. The operation was performed at moderate hypothermia (28°C). Myocardial protection was achieved with topical ice saline solution and cold blood cardioplegia infused directly through both coronary ostia.
The leaflets of the aortic valve were examined. The free edges of the leaflets were thin and mobile. The ascending aorta was completely transected immediately above the sinotubular junction and just below the aortic crossclamp. A 28 mm Dacron graft was used to perform supracoronary ascending aortic replacement. The crossclamp time was 24 minutes, and weaning from cardiopulmonary bypass did not require any inotropic support. Following sternal closure, plastic surgeons performed a unilateral partial pectoralis major myocutaneous flap transpositon to cover the defect over the sternum. The postoperative course was uneventful and the patient was discharged after 10 days.
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DISCUSSION
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The correct timing of operation for patients with AAA is clinically established and surgeons can choose from a large spectrum of surgical approches. The most serious complications of an AAA are rupture, and dissection, which are almost invariably fatal. The rate of ruptures and dissections is significantly higher in patients with a higher initial aortic size. In the classical surgical era, the accepted upper limit for an ascending aortic aneurysm was 5.5 cm with an average growth rate of 0.42 cm per year.1 However, most surgeons now use a smaller limit of 5 cm in patients with Marfan’s disease, or those with a family history of the disease. Our experience has shown that Non-Marfan’s patients also manifest a familial clustering of aortic aneurysms and dissections. Using genetic analysis, patients revealing 21% of probands with aortic aneurysms have been shown to have a first-order relative with a known or likely aortic aneurysm. In cases with a familial form of aortic aneurysm, early surgical intervention should be considered to prevent rupture and dissection.2
The literature reveals only a small number of cases involving the combination of both malignant sternal tumors and aortic aneurysms.3,4 The underlying causal relationship between the occurrence of aortic aneurysms and chest wall tumors is not well-known. In addition, this is the first case reported involving the combination of these 2 important pathologies. Treatment options for these patients should weigh the risk of complications caused by the dilated aorta and a malignant tumor against the risk of complications from the one-stage operation.
This case describes an unusual presentation of this combination with some challenging aspects. The most critical aspect for this patient was the correct timing of operation to prevent any catastrophic complications arising from the dilated aorta. In the absence of a soft tissue tumor, this patient would not be a surgical candidate but closely monitored instead by routine radiological examinations. The presence of fibrosarcoma complicated the picture as the patient also required a complete resection and muscle transposition procedure as soon as possible. After such a reconstructive procedure, a reasonable period of time is necessary to allow adherence of the myocutaneous flap to the underlying tissue. However, because of the patient’s aortic pathology, elective or even emergent surgical intervention may be necessary during this time. We concluded that postponing aortic replacement after the muscle transposition procedure would put the patient at risk. As such, the one-stage operation was elected as the treatment of choice.
Fibrosarcomas are uncommon malignancies usually originating wherever fibrous tissue is found and are potentially curable by surgical excision.5 In our patient, we did not institute neoadjuvant or adjuvant treatment modalities because the tumor was amenable to complete en bloc resection and reconstruction. The absence of the involvement of deeper structures obviates the need for a more extensive procedure and the operation should be carried out through a standard median sternotomy.
Ascending aortic aneurysms are among the most common potentially fatal aortic diseases requiring urgent surgical intervention to prevent aortic rupture or dissection. The combination of ascending aortic aneurysm and suprasternal fibrosarcoma is rarely reported in the literature. With the recent refinements in cardiac anaesthesia and surgical techniques, the surgical repair of ascending aortic aneurysms associated with suprasternal masses can be accomplished in a one-stage operation with a short hospitalization period and low operative mortality.
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REFERENCES
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ABSTRACT
INTRODUCTION
