Ann Thorac Surg 1997;63:1766-1768
© 1997 The Society of Thoracic Surgeons
Case Report
Osteogenic Sarcoma of the Left Atrium
Surajit Dan, MCh,
Andrew J. Hodge, FRACS
Department of Cardio-thoracic Surgery, The Mount Hospital, Perth, Australia
Accepted for publication January 13, 1997.
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Abstract
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A primary cardiac osteogenic sarcoma is described in a patient presenting with a left atrial obstructive lesion. Wide surgical excision was possible with left atrial reconstruction and mitral valve replacement. The patient survived the operation and was symptom free for 1 year, finally dying at 18 months of cerebral metastases.
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Introduction
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Primary sarcomas of the heart are rare and constitute less than 25% of cardiac tumors [1]. Most articles are restricted to case reports or case examples. Among cardiac sarcomas, osteogenic sarcoma is an even more rare entity. A 1992 review by Burke and associates [2] from the Armed Forces Institute of Pathology found only 9 cases. We report a further case of cardiac osteosarcoma from our experience.
A 32-year-old female patient presented with right hemiparesis, preceded by a transient ischemic attack in the same cerebral territory. On echocardiogram, a small mass on the posterior leaflet of the mitral valve was passed off as a vegetation due to bacterial endocarditis. The chest roentgenogram at this stage was entirely normal. The right hemiparesis gradually resolved over 2 months. After 6 months, due to recurrent palpitation, the echocardiography was repeated and revealed a large tumor in the posterior part of the left atrium together with marked thickening of both cusps of the mitral valve and possible involvement of the interatrial septum (Fig 1
). Chest roentgenography now showed a pleurally based mass in the left upper zone. Clinical examination demonstrated thickening of the right deltoid muscle, not previously present. It was assumed given this sequence of events that the primary tumor was cardiac, with pleural and muscular metastasis.
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Fig 1. . Echocardiogram showing tumor (T) in left atrium (LA), attached to mitral valve. (Ao = aorta; LV = left ventricle.)
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An urgent cardiac operation was undertaken. The patient was placed on cardiopulmonary bypass by aortic, mid-superior vena caval, and femoral venous cannulation, the latter cannula being advanced into the inferior vena cava. Myocardial protection was achieved by core cooling to a pharyngeal temperature of 28°C, and antegrade and retrograde blood cardioplegia given at 30-minute intervals. The myocardial temperatures ranged in both ventricles between 12° and 20°C.
The left atrium was opened through the roof and the tumor was found to have two portions: a large posterior mass involved the majority of the interatrial septum, extending down the septum across the posterior wall and across the mitral annulus into almost the whole of the posterior cusp. This mass did not encroach on the orifices of the pulmonary veins, but invaded the left atrial wall adjacent to the orifices of both inferior veins. The second portion was a 1.5-cm sessile mass on the upper surface of the anterior cusp with a further extension to the anterior annulus.
The left atrial incision was extended to the right with division of the superior vena cava 2 cm above its termination. The interatrial septum was excised, and removal of the tumor from the lower posterior wall of the left atrium required entering the orifice of the right inferior pulmonary vein, taking the majority of the wall between the pulmonary vein orifices, and going right to the lip of the left inferior pulmonary vein. The posterior mitral annulus required removal together with the posterior cusp. The anterior cusp was then removed together with the adjacent portion of anterior annulus and left atrial wall, the latter being repaired by direct suture. The atrium was then reconstructed with a large patch of autologous, nontreated pericardium, which was sutured into the defect, creating a new posterior wall and interatrial septum (Fig 2). The superior vena cava was reconnected with a patch of pericardium to prevent local distortion. Division and resuture of the inferior vena cava was required to expose a posterior defect in the neo-left atrial wall for hemostasis. The mitral valve was replaced with a 31-mm St Jude Medical (St. Paul, MN) mechanical prosthesis. The patient required cardiopulmonary bypass for 365 minutes and an aortic cross-clamp time of 301 minutes.
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Fig 2. . Schematic diagram of left atrium showing the area of resection, which was later reconstructed with pericardium. (MV = mitral valve; PV = pulmonary vein.)
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The left mediastinal pleura was divided to explore the pleurally based mass in the left chest. Biopsy of this mass was performed, but the mass could not be excised due to extensive chest wall invasion. A biopsy specimen was also taken from the right deltoid muscle. Pathologic examination of the left atrial tissue revealed primary cardiac osteosarcoma with elements of mesenchymoma (Figs 3, 4). The left chest mass and the deltoid mass were considered to be metastases and revealed the same tissue type.
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Fig 4. . Microscopic photograph of tumor showing malignant osteoid formation. (Hematoxylin and eosin; x330 before 54% reduction.)
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Postoperatively the patient had a transient period of amnesia, brain computed tomographic scan revealing focal low attenuated areas in the basal ganglia and the posterior limb of the internal capsule, which were considered to be possible secondary tumors from the primary cardiac tumor. She received a complete course of chemotherapy over 6 months with doxorubicin and cisplatin alternating with cyclophosphamide and VP16. With chemotherapy the chest wall mass diminished in size and the mass in the right deltoid muscle also improved significantly. The residual mass in the right shoulder was treated with local radiotherapy with nearly complete disappearance. Six months after the operation, echocardiography was repeated, which showed no tumor mass in any cardiac chamber, and she remained in New York Heart Association class I. Two months after completion of chemotherapy a symptomatic cerebral secondary tumor developed in the parietal lobe. The chest wall mass recurred with left axillary node enlargement. A further course of chemotherapy was not given with consideration of the patient's clinical condition and bleak prognosis. The patient died 1
years after initial presentation.
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Comment
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Cardiac osteosarcoma is a clinical and pathologic surprise [3]. Possible clinical presentations are similar to those of other mass lesions in the cardiac chambers: obstructive, embolic, or metastatic [4]. These tumors are more common in male patients [2]. Because they are highly aggressive, with local invasion of surrounding vital structures and early metastasis, the average survival is 6 months from the time of diagnosis [5]. Operation with wide excision is the primary mode of treatment [6]. Some investigators [1, 7] consider resection only as a palliative measure, the goal of which is to relieve symptoms of obstruction, but long-term survival of these patients also has been reported [6]. Postoperative chemotherapy is useful palliation [8].
This case demonstrates that removal of the majority of the left atrial structures is possible, with very satisfactory functional results after simple reconstruction.
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Footnotes
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Address reprint requests to Dr Hodge, Kirkman House, 10 Murray St, Perth, Western Australia 6000, Australia.
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References
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- Silverman NA. Primary cardiac tumors. Ann Surg 1980;191:127–38.[Medline]
- Burke A, Cowan D, Virmani R. Primary sarcoma of heart. Cancer 1992;69:387–94.[Medline]
- Burke AP, Virmani R. Osteosarcomas of the heart. Am J Surg Pathol 1991;51:289–95.
- Gudjonsdottir A, Hagerstrand I. Primary sarcoma of the heart: a case report. Acta Pathol Microbiol Scand (A) 1971;79:604–6.
- Dein JR, Frist WH, Stinson EB. Primary cardiac neoplasms: early and late results of surgical treatment in 42 patients. J Thorac Cardiovasc Surg 1987;93:502–11.[Abstract]
- Murphy MC, Sweeney MS, Putnam JB Jr, et al. Surgical treatment of cardiac tumours-a 25 year experience. Ann Thorac Surg 1990;49:612–7.[Abstract/Free Full Text]
- Chitwood WR Jr. Cardiac neoplasms: current diagnosis, pathology, and therapy. J Cardiac Surg 1988;3:119–54.[Medline]
- Ueda T, Aozasa K, Tsujimoto M, et al. Multivariate analysis for clinical prognostic factors in 163 patients with soft tissue sarcoma. Cancer 1988;62:1444–50.[Medline]
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Abstract
Introduction
