Ann Thorac Surg 2000;70:1412-1414
© 2000 The Society of Thoracic Surgeons
Case report
Complete resection of cardiac leiomyosarcoma extending into the pulmonary trunk and right pulmonary artery
Tetsuya Kono, MDa,
Takahiro Takemura, MDa,
Ikuo Hagino, MDa,
Goki Matsumura, MDa
a Department of Cardiovascular Surgery, National Nagano Hospital, Ueda, Japan
Address reprint requests to Dr Kono, Department of Cardiovascular Surgery, National Nagano Hospital, Midorigaoka 1-27-21, Ueda 386-8610, Japan
e-mail: tetsuya{at}hsp.md.shinshu-u.ac.jp
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Abstract
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A cardiac leimyosarcoma is an extremely rare tumor. We report a case of complete resection of a cardiac leiomyosarcoma extending into the pulmonary trunk and the right pulmonary artery using the Freestyle bioprosthesis (Medtronic, Inc, Minneapolis, MN) and Xenomedica graft (Baxter Healthcare Corp, Horw, Switzerland). Extensive resection and reconstructive surgery with the addition of radiotherapy prolonged the patient’s life.
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Introduction
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Acardiac leimyosarcoma is an extremely rare tumor [1] and shows a poor prognosis [2]. These tumors are highly malignant and locally invasive, thus complete resection is often impossible. Complete resection using alloplastic [3] or homograft valve [4] replacements of the pulmonary valve has been reported in only a few cases. We report a case of complete resection of a cardiac leimyosarcoma extending into the pulmonary trunk and the right pulmonary artery using the Freestyle bioprosthesis (Medtronic, Inc, Minneapolis, MN) and Xenomedica graft (Baxter Healthcare Corp, Horw, Switzerland). Adjuvant radiotherapy was carried out after the operation, and the patient is alive without evidence of recurrence 11 months after the operation.
A 37-year-old man had dyspnea and faintness. His medical history was not remarkable. He was free of cardiovascular disease or other symptoms until dyspnea developed. On admission, he looked physically ill. On auscultation, the first and second sounds revealed normal intensity, and a grade 4 systolic murmur was audible along the left sternal border. The abdomen was soft and flat, and the liver was not palpable.
The results of the laboratory examination on admission were almost normal. Chest roentgenograms disclosed no cardiomegaly and a cardiothoracic ratio of 42%. Transthoracic two-dimensional echocardiography revealed a large mass extending from the ventricular septum to the right ventricular outflow tract and the right pulmonary artery, with enlargement of the right ventricle and oppression of the ventricular septum toward the left ventricle. Doppler echocardiographic measurements demonstrated a maximum pressure gradient of 67 mm Hg across the right ventricular outflow tract.
Right heart catheterization showed a mean right atrial pressure of 10 mm Hg and a right ventricular pressure of 90/12 mm Hg. Coronary arteriography revealed two feeding arteries from the conus branch of the right coronary artery and the left anterior descending artery. Right ventriculography showed a large filling defect in the right ventricular outflow tract. Magnetic resonance imaging disclosed a solid mass in the right ventricular outflow tract and wall thickening of the pulmonary trunk (Fig 1). There was no evidence of local chest wall invasion or enlarged mediastinal lymph nodes.
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Fig 1. Magnetic resonance imaging disclosed a solid mass (arrowheads) in the right ventricular outflow tract (right) and wall thickening (arrowheads) of the pulmonary trunk (left).
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Because of intracirculatory tumor growth and the absence of distant metastasis, a radical tumor resection was proposed. A lower mini-sternotomy was performed, and cardiopulmonary bypass was established. The tumor originated from the ventricular septum and almost completely blocked the lumen of the right ventricular outflow tract. In addition, the tumor invade the main pulmonary artery and anterior wall of the right pulmonary artery. The tumor was resected including the ventricular septum and the right ventricular outflow tract. The main pulmonary artery was resected including the pulmonary valve, and the right pulmonary artery was also resected underneath the superior vena cava. The resected ventricular septum was patch repaired, and the resected outflow tract of the ventricle and pulmonary artery was reconstructed with a Freestyle bioprosthesis and Xenomedica graft, respectively (Fig 2). Perioperative biopsy specimens of the edge were free of tumors.
Pathologic analysis of the resected specimen revealed that the nuclei of the tumor cells varied greatly in size, shape, and chromaticity, and the specimen was diagnosed as a leiomyosarcoma. Although the patient suffered from low cardiac syndrome that required mechanical circulatory support after the operation, he was discharged on postoperative day 94. Adjuvant radiation therapy was performed, and he is alive without evidence of recurrence 11 months after the operation.
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Comment
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Malignant cardiac tumors are rare diseases. Most of these tumors are sarcomas, fibrosarcomas, and liposarcomas. A primary cardiac leiomyosarcoma is very rare and constitutes less than 0.2% of all cardiac tumors [1]. There has been only one case among 133 surgically treated patients reported from the Texas Heart Institute by Murphy and associates [5].
Previously, most patients were diagnosed postmortem, but advances in echocardiography, computed tomography, and magnetic resonance imaging have made it possible to diagnose this disease in living patients. The most useful diagnostic method of an intracardiac tumor is two-dimensional echocardiography.
The mean survival time of patients with leiomyosarcoma is 6 months after diagnosis [2]. These tumors are highly aggressive and locally invasive; therefore, resection is often incomplete because of the advancement of the tumors, and the aims of the surgical procedure are at relieving the symptoms of cardiac chamber, valve, or major blood vessel obstructions. In our patient, because of the absence of distant metastasis and intracirculatory tumor growth, a radical tumor resection was performed. The resected ventricular septum was patch repaired. In addition, the resected outflow tract of the ventricle and pulmonary artery was reconstructed using the Freestyle bioprosthesis and Xenomedica graft, respectively. A few cases of alloplastic [3] or homograft valve [4] replacement have been reported in the literature.
Combined chemotherapy and/or radiation therapy after resecting a tumor is somewhat effective, and some patients have survived more than 2 years after operation with radiation and/or chemotherapy [6]. Therefore, we performed adjuvant radiotherapy. He was discharged and is alive without evidence of recurrence 11 months after the operation.
In conclusion, extensive resection of a leiomyosarcoma and reconstructive surgery using the Freestyle bioprosthesis and Xenomedica graft with the addition of radiotherapy can prolong a patient’s life.
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References
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Kirklin JW, Barratt-Boyes BG. Cardiac surgery, New York: Churchill Livingstone, 1986:1636.
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Antunes M.J., Vanderdonck K.M., Andrade C.M., Rebelo L.S. Primary cardiac leiomyosarcomas. Ann Thorac Surg 1991;51:999-1001.[Abstract/Free Full Text]
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Tanaka I., Masuda R., Inoue M., et al. Report of a case with complete resection and graft replacement, and review of 47 surgically treated cases reported in the literature. Thorac Cardiovasc Surg 1994;42:64-68.[Medline]
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Zerkowski H.R., Hofmann H.S., Gybels I., Knolle J. Primary sarcoma of pulmonary artery and valve. J Thorac Cardiovasc Surg 1996;112:1122-1124.[Free Full Text]
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Murphy M.C., Sweeney M.S., Putnam J.B., Jr, et al. Surgical treatment of cardiac tumors; a 25-year experience. Ann Thorac Surg 1990;49:612-618.[Abstract/Free Full Text]
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Okabayashi H., Fujiwara Y., Kanzaki Y., Mitsudou K., Yamamoto H. A case report of primary leiomyosarcoma of the heart. Jpn J Thorac Surg 1987;35:2161-2165.
Accepted for publication April 25, 2000.
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Abstract
Introduction
