Ann Thorac Surg 1998;66:1810-1811
© 1998 The Society of Thoracic Surgeons
Case Reports
Six-year survival after excision of cardiac malignant lymphoma
Masatake Takagi, MDa,
Toshiyasu Kugimiya, MDa,b,c,
Yoshiyuki Miyahara, MDa,b,c,
Tomayoshi Hayashi, MDa,b,c
a Department of Cardiovascular Surgery, Nagasaki University School of Medicine, Nagasaki, Japan
b Department of Medicine, Nagasaki University School of Medicine, Nagasaki, Japan
c Department of Pathology, Nagasaki University School of Medicine, Nagasaki, Japan
Accepted for publication May 6, 1998.
Address reprint requests to Dr Takagi, Department of Cardiovascular Surgery, Nagasaki University School of Medicine, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan
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Abstract
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We report a patient with a postoperative survival period of 6 years after the surgical excision of a cardiac malignant lymphoma. A 35-year-old woman underwent total excision of the tumor arising from the left ventricular outflow tract. After the operation, she was treated with chemotherapy for 6 months. She has been doing well thereafter without any medication. To date there is no evidence of recurrence.
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Introduction
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A 35-year-old woman was found to be positive for anti-human T lymphotropic virus-I antibody and was diagnosed as having pre-adult T cell leukemia-lymphoma (pre-ATL) in October 1987. Although abnormal lymph cells accounted for 5% to 6% of the total lymph cells in the peripheral blood, she did not undergo chemotherapy. In December 1989, mild exertional dyspnea occurred but she still received no treatment until March 1991. In April 1991, an abnormal mass was found in her left ventricular outflow tract by echocardiography. She was then referred to our department. On admission, cyanosis was observed in her lips and nails and she complained of general fatigue and dizziness. Generalized lymph node swelling was observed and a grade 3/6 systolic murmur was recognized.
A chest roentgenogram revealed cardiomegaly and moderate pulmonary congestion with a cardiothoracic ratio of 0.60. A solid mass was observed in the left ventricular outflow tract immediately below the aortic valve by long-axis–view echocardiography. The mass moved across the aortic valve to the sinus of Valsalva during systole (Figs 1, 2). The data obtained by cardiac catheterization were as follows (in millimeters of mercury): mean right atrial pressure, 3; pulmonary arterial pressure, 23/9 (mean, 14), mean pulmonary artery wedge pressure, 9; right ventricular pressure, 25/0 to 4; left ventricular pressure, 179/0 to 7; and aortic pressure, 107/64. The serologic examination revealed positive anti-human T lymphotrophic virus-I antibody, and negative anti-human immunodeficiency virus antibody.
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Fig 1. Parasternal long-axis plane image of the echocardiography shows a solid mass (arrow) in the left ventricular outflow tract.
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Fig 2. Left ventricular cineangiogram shows a filling defect (arrows) at the left ventricular outflow tract.
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On May 7, 1991, the patient underwent an operation under cardiopulmonary bypass and moderate hypothermia. A 2 x 2-cm tumor mass, which was found in the sinus of Valsalva, moved from the left ventricular outflow tract via the aortic valve (Fig 3). This tumor originated from the ventricular septum below the left and right coronary cusps of the aortic valve and was just adjacent to the aortic valve. The border between the tumor and the aortic valve was apparent. The tumor was excised widely along with the surrounding tissues including normal endocardium in the ventricular septum, leaving the aortic valve intact. The pathologic findings of the excised tissues were diffuse lymphoma pleomorphic LCA (+), pan T (+), pan B (+), CD3 (+), CD4 (+), and CD25 (Tac) (-) (Fig 4), and the patient was diagnosed as having malignant lymphoma.
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Fig 3. The tumor, which was found in the sinus of Valsalva, moved from the left ventricular outflow tract.
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Fig 4. Histopathologic view of the tumor. There are diffuse lymphoid cells. The tumor cells show marked convoluted nuclei. There are also large pleomorphic cells with convoluted nuclei. (Hematoxylin and eosin; x1,000 before 53% reduction.)
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The postoperative course was satisfactory. Chemotherapy with vincristine, doxorubicin, etoposide, prednisolone, and pentostatin was performed for 6 months after the operation. Since the completion of that chemotherapy, no further chemotherapy has been performed, and the patient was doing well without any sign of recurrence as of April 1998.
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Comment
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Surgical treatment of cardiac malignant lymphoma, both primary and metastatic, usually does not obtain satisfactory results [1, 2]. The occurrence of a tumor (whether malignant or benign) in the left ventricular outflow is rare. Malignant lymphoma is sometimes accompanied by acquired immunodeficiency syndrome or occurs after heart transplantation [3, 4]. Adult T cell leukemia-lymphoma is also sometimes accompanied by malignant lymphoma or lymphoma. The present patient was positive for anti-human T lymphotrophic virus-1 antibody, although symptomatic ATL had not developed, suggesting a relationship between ATL and the occurrence of malignant lymphoma [5]. Although the tumor was partially connected with the base of the left coronary cusp of the aortic valve, the border between the tumor and aortic valve was relatively clear. Because ATL is often accompanied by a hemorrhagic tendency, we avoided valve replacement in this patient. Although the results of chemotherapy for malignant lymphoma are improving, patients still sometimes die of infectious complications before complete remission. We experienced a patient with cardiac primary malignant lymphoma who died of pneumonia when the lymphoma was almost cured [6]. In the present case, the patient’s young age, good response to chemotherapy, and the fact that ATL had not yet appeared even though the patient was positive for anti-human T lymphotropic virus-1 antibody may be the reasons for the very long survival period.
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References
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- Hake U., Iversen S., Schmid F.X., Erbe R., Oelert H. Urgent indication for surgery in primary or secondary cardiac neoplasm. Scand J Thorac Cardiovasc Surg 1989;23:111-114.[Medline]
- Holladay A.O., Siegel R.J., Schwartz D.A. Cardiac malignant lymphoma in acquired immune deficiency syndrome. Cancer 1992;70:2203-2207.[Medline]
- Burtin P., Guerci A., Boman F., et al. Malignant lymphoma in the donor heart after heart transplantation. Eur Heart J 1993;14:1143-1145.[Abstract/Free Full Text]
- Sakashita A., Hattori T., Miller C.W., et al. Mutation of the p53 gene in adult T-cell leukemia. Blood 1992;79:477-480.[Abstract/Free Full Text]
- Takagi M., Kugimiya T., Fujii T., et al. Extensive surgery for primary malignant lymphoma of the heart. J Cardiovasc Surg 1992;33:570-572.[Medline]
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Abstract
Introduction
