Ann Thorac Surg 1996;62:1526-1528
© 1996 The Society of Thoracic Surgeons
Case Report
Primary Tumors of the Aorta
Ashish Kumar Das, MD,
Konda Subba Reddy, MD,
Pan Suwanjindar, MD,
Alison Fulmer, MD,
Carmelindo Siquiera, Jr, MD,
H. Storm Floten, MD,
Albert Starr, MD
St. Vincent Hospital, Portland, Oregon
Accepted for publication June 8, 1996.
 |
Abstract
|
|---|
Primary tumors of the aorta are rare and are usually malignant. Thirty-three cases are reported in the world literature, of which 2 are myxomas. The diagnosis is difficult and is commonly made postoperatively or at autopsy. We present a case of intraluminal aortic myxoma that presented as recurrent arterial emboli and was managed successfully. This case showed the sensitivity of magnetic resonance imaging and transesophageal echocardiography in diagnosis.
|
Introduction
|
|---|
The first known case of aortic tumor was described by Brodowski [1] in 1873. Since then only 33 cases of primary aortic tumors have been documented in the literature. These tumors are of varying histologic types. They characteristically present with diverse clinical manifestations, which preclude accurate presumptive or antemortem diagnosis. Most of the diagnoses have been made after operation [2] or at postmortem examination [3]. The underlying common denominators, however, are major vascular obstruction and peripheral embolization. Magnetic resonance imaging and transesophageal echocardiography may suggest the diagnosis preoperatively and help in making an appropriate plan of therapy.
A 44-year-old woman presented with sudden onset of pain in her left calf. There was no prior history of claudication, palpitation, heart disease, or hypercoagulability. Her left leg was cold. Dorsalis pedis and posterior tibial pulses were absent, whereas the popliteal pulse was normal. The clinical diagnosis of acute arterial occlusion was made. Clinical examination of chest, cardiovascular system, and abdomen was essentially normal. The arteriogram revealed thrombosis of distal anterior and posterior tibial arteries. She was treated with intraarterial urokinase followed by intravenous heparin. She was discharged with warfarin. The hypercoagulability screen was negative. Transesophageal echocardiography revealed normal chambers, no evidence of thrombus, intact interatrial septum, normal left ventricle, and normal valves. There was a mobile, echo-dense area approximately 0.5 cm in size in the descending thoracic aorta (Fig 1
).
View larger version (97K):
[in this window]
[in a new window]
|
Fig 1. . Transesophageal echocardiogram showing a pedunculated, highly mobile, echogenic mass with a gelatinous appearance and variable shape during the cardiac cycle (transverse view of the aorta).
|
|
Computed tomography of the chest and abdomen with contrast enhancement did not image any abnormality.
Magnetic resonance imaging (Fig 2) identified a small, abnormal soft tissue mass adjoining the anterior wall of the aorta approximately 3.5 cm caudal to the origin of the left subclavian artery. The lesion measured 5 mm anterioposteriorly, 6 mm transversely, and 10 mm craniocaudally and appeared to be smaller than the one on initial transesophageal echocardiography. We decided to follow her case because magnetic resonance imaging findings were suggestive of aortic thrombus. Serial follow-up echocardiograms over the next 6 months did not show any significant change. During this period, she had another episode of peripheral arterial embolization.
View larger version (124K):
[in this window]
[in a new window]
|
Fig 2. . (A) A small, abnormal, round soft tissue mass adjoining the anterior wall of the proximal aorta (magnetic resonance imaging scan, axial view). (B) Oblique coronal view showing abnormal soft tissue mass in the proximal aorta.
|
|
With the presumptive diagnosis of primary aortic tumor, operative removal was planned. The patient was placed in the right lateral decubitus position with 30-degree tilt to the left. Left femorofemoral bypass was established. Thoracotomy was performed through the bed of resected fifth rib.
The aorta was normal externally and was relatively free from atherosclerosis. The aorta was cross-clamped proximal to the left subclavian artery and at the level of the T-6 vertebra distally. The aorta was opened. The origin of the ligamentum arteriosum could easily be identified. A centimeter from the ligamentum arteriosum a pedunculated, very fragile mass that looked myxomatous was visible. It did not invade the aortic wall but was intimately attached to the aortic intima. The intimal base was removed as a disc approximately 10 mm in diameter, including the media, leaving the adventitia behind. The defect in the aorta was closed with a double layer of continuous 5-0 Prolene (Ethicon, Somerville, NJ). The patient tolerated the procedure very well and was discharged from the hospital on the fifth postoperative day.
The histopathologic features (Fig 3) of the surgical specimen were consistent with myxomatous intraluminal aortic tumor.
View larger version (128K):
[in this window]
[in a new window]
|
Fig 3. . Section of the aortic lesion showing stellate, spindle-shaped cells in the myxoid stroma. (Hematoxylin and eosin; x250 before 53% reduction.)
|
|
|
Comment
|
|---|
The diagnosis of primary aortic tumor is difficult because of its diverse manifestations. These tumors clinically may mimic heterogeneous conditions such as dissecting aneurysm; coarctation of aorta; intrathoracic, abdominal, and retroperitoneal malignancies; and peripheral arteriosclerotic vascular disease [4]. Aortic tumors are more often diagnosed at operation or at autopsy, and most of them are histologically malignant [5]. A review of the world literature found 33 previously reported cases of primary aortic tumors (Table 1). The sites of origin of the tumors are distributed over the various aortic segments in the following way: 9 from thoracic aorta, 13 from abdominal aorta, and 12 from thoracic and abdominal aorta [6]. Of the 34 cases including the present case, there have been 21 male and 13 female patients; age ranged from 16 to 75 years. Only five tumors were nonmalignant and 29 were malignant [7]. Wright and associates [8] classified aortic tumors on the basis of their site of origin within the aortic wall: (a) tumors involving primarily the intima and (b) tumors arising from media and adventitia. Intraluminal tumors frequently give rise to thromboembolism, whereas those arising from the media and adventitia form aggressive mass lesions, which may have intraluminal and extraluminal extension.
As a rule, if the echocardiogram does not show any thrombus in the cardiac chambers, the possibility of aortic thrombus must be considered. Computed tomography cannot differentiate intraluminal tumor from aortic thrombus, but it can exclude extraaortic mass. Magnetic resonance imaging has an advantage over combined aortography and computed tomography. With magnetic resonance imaging the displacement of flowing blood provides inherent contrast between vessel lumen and vessel wall or surrounding soft tissue. In addition, magnetic resonance imaging provides an oblique view of the aorta, which can be helpful in diagnosing aneurysms, dissections, and other vascular pathologies. Magnetic resonance imaging also has an advantage over arteriography because the risks of tumor embolization and contrast reaction are eliminated. Similarly, transesophageal echocardiography showed excellent accuracy in diagnosing and determining the extent and location of the aortic tumor in this case (see Fig 1
).
The therapeutic management of the aortic tumor is unclear. Surgical methods such as primary resection with reconstruction are practically feasible in benign tumors. In malignant tumors the survival has been very poor [5]. Thus, the management is individualized. The tumors are inoperable in the majority of cases. Excision and replacement with a tube graft, with or without some kind of bypass, is recommended if it is possible.
The myxoma reported here is a very localized tumor with no invasion of the media or adventitia. Although the tumor shows certain resemblance to cardiac myxoma, soft tissue myxoma certainly results in local infiltration.
|
Acknowledgments
|
|---|
We thank Jennifer Bolger for her help in the preparation of the manuscript.
|
Footnotes
|
|---|
Address reprint requests to Dr Das, Albert Starr Academic Center, 9155 SW Barnes Rd, Suite 240, Portland, OR 97225.
|
References
|
|---|
- Brodowski W. Primares sarkom der aorta thoracicamit verbreitung des neugebildes in der unteren korperhalfte. Jahresber Leistungen Fortschr Gesammten Med 1873;8:243–6.
- Gough J., Moreano W. Primary myxoma of the aorta. Clin Pathol 1974;27:806–7.
- Karl T, Chen K. Primary malignant fibrous histiocytoma of the aorta. Cancer 1981;48:840–4.[Medline]
- Mason MD, Wheeler JR, Gregory RT, et al. Primary tumors of the aorta: report of a case and review of the literature. Oncology 1982;39:167–72.[Medline]
- Fonseca JL, Fernandez-Valderrama I, Gesto R, et al. Malignant fibrous histiocytoma of the aorta complicated by anuria. Ann Vasc Surg 1992;6:164–7.[Medline]
- Schipper J, van Oostayen JA, den Hollander JC, van Seyen AJ. Aortic tumors: report of a case and review of the literature. Br J Radiol 1989;62:35–40.[Abstract]
- Ronaghi AH, Roberts AC, Rosenkrantz H. Intra-aortic biopsy of a primary aortic tumor. J Vasc Intervent Radiol 1994;5:777–80.[Medline]
- Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985;9:890–7.[Medline]
Top
Abstract
Introduction
