Ann Thorac Surg 1998;66:553-555
© 1998 The Society of Thoracic Surgeons
Case Reports
Successful removal of massive cardiac neurilemoma with cardiopulmonary bypass
Takehisa Hashimoto, MDa,
Shoji Eguchi, MDa,
Takashi Nakayama, MDa,
Hajime Ohzeki, MDa,
Jun-ichi Hayashi, MDa
a Department of Thoracic and Cardiovascular Surgery, Niigata University School of Medicine, Niigata, Japan
Accepted for publication February 23, 1998.
Address reprint requests to Dr Hashimoto, Department of Thoracic and Cardiovascular Surgery, School of Medicine, Niigata University, 1—751 Asahimachi-dori, Niigata City, 951, Japan
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Abstract
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A 46-year-old woman was referred to our hospital because of cardiac enlargement seen on a chest radiograph. Imaging studies showed a massive intrapericardial tumor with a size of 12x8x7 cm. Tumor dissection included inspection of the inner aspect of the superior vena cava with use of cardiopulmonary bypass, because the mass was tightly adherent to both superior vena cava and right atrium. The pathologic diagnosis was neurilemoma.
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Introduction
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Primary neurogenic neoplasms of the heart are extremely uncommon. Neurilemoma, a benign tumor, is among the rarest [1, 2]. To our knowledge, only 7 cases of cardiac neurilemoma have been reported [1–7]. We present the case of a large cardiac neurilemoma, which was removed successfully with use of cardiopulmonary bypass.
A 46-year-old woman was admitted to our hospital for investigation of an enlarged cardiac silhouette seen on a chest radiograph. Past surgical history included a uterine myomectomy. Examination on admission revealed slight dilation of both external jugular veins and a grade 2/6 systolic murmur at the left sternal border. The electrocardiography showed regular sinus rhythm. A chest roentgenogram revealed cardiomegaly with a cardiothoracic ratio of 79%. Echocardiography confirmed a mass measuring 7x8 cm with a location superficial and anterior to the right atrium and an inhomogeneous echo density. Mild mitral and tricuspid regurgitation, and a small pericardial effusion also were evident. Computed tomography (Fig 1) and magnetic resonance imaging revealed a massive solid intrapericardial tumor between the superior vena cava (SVC) and ascending aorta. The caval wall was indented toward the right and increased in thickness. An angiogram showed two feeding arteries originating from both the circumflex and right coronary arteries. A pressure gradient of 7 mm Hg was measured between the SVC and the right atrium. Arrangements were made for immediate operation in anticipation of a malignant tumor or cardiac complication.
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Fig 1. Computed tomography revealed a solid mass (arrow) measuring 9x9x8 cm located between the right atrium, superior vena cava (SVC), ascending aorta (Ao), and right pulmonary artery (PA).
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At operation, we prepared cardiopulmonary bypass in anticipation of tumor invasion of a cardiac chamber or the great vessels. Through a median sternotomy and pericardiotomy, 200 mL of yellowish pericardial fluid was aspirated. Cytologic examination demonstrated no malignant cells. The tumor was located in close relation to the right atrium, SVC, azygos vein, ascending aorta, left atrium, and right pulmonary artery. To avoid phrenic nerve injury, we opened the right pleural space and retracted it. Tight adhesion to the right atrium and SVC necessitated cardiopulmonary bypass. It was easy to cannulate the SVC above this tumor because the SVC was elongated and the space for cannulation was enough. The SVC was divided, and the tumor was dissected carefully with ongoing inspection from the internal aspect of the SVC. The SVC was reconstructed with an end-to-end anastomosis. Macroscopically the tumor was encapsulated, not invasive, and sure to have a benign nature, so a frozen section was not done at the time of the operation.
The tumor was surrounded by a thin fibromembranous capsule, measured 12 x 8 x 7 cm, and weighed 360 g. Its cut surface revealed both cream-colored and yellow areas, with secondary degenerative changes such as cystification, calcification, and focal hemorrhage. Microscopic sections showed a mixed proliferation of typical Antoni type A and Antoni B tissue. Immunohistochemical staining was positive for S-100 protein. These findings resulted in a diagnosis of neurilemoma, a benign tumor.
The patient had normal phrenic nerve function and rapidly recovered from the operation. A subsequent angiogram showed a smooth internal surface where the SVC had been reconstructed. No evidence of recurrence was noted 2 years after the operation.
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Comment
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Neurilemoma typically involves the flexor surfaces of extremities, neck, mediastinum, retroperitoneum, posterior spinal roots, or cerebellopontine angle [2]. Although neurogenic tumors make up 33% of mediastinal tumors [8], primary neurogenic neoplasms of the heart are extremely uncommon. To our knowledge, only 7 cases of cardiac neurilemoma have been reported [1–7]. Most of these tumors have been right-sided, arising near the cardiac plexus [1–3, 6, 7], with 2 cases occurring on the left [4, 5]. Primary neurogenic neoplasms of the heart arise from cardiac branches of the vagus nerve and the cardiac plexus [2, 6]. We believe the present lesion originated from the cardiac plexus extending from the right atrial wall to the SVC, ascending aorta, and right pulmonary artery.
The massive cardiac neurilemoma seen in our patient compressed the SVC and appeared to increase its thickness; it had no symptoms or signs except for slight jugular venous dilatation and elevation of the SVC pressure. Because benign cardiac neoplasms may compress or occupy the cardiac chambers, their manifestations are not type-specific but rather an effect of their size and location [5, 6]. Cardiovascular symptoms of such a tumor may include chest pain, syncope, heart failure, cardiac tamponade, arrhythmias, and conductive disorders. Chronic obstruction may cause cardiac failure, with intermittent obstruction representing one cause of syncope. A right-sided tumor may produce obstruction of the SVC [6]. Among the reported occurrences of cardiac neurilemoma, two right-sided tumors have produced right ventricular outflow tract obstruction and subsequent dyspnea [3, 6], and a left-sided tumor has caused atrial fibrillation [4].
Clinical differentiation of cardiac neurilemoma from other cardiac tumors is impossible except by histologic examination. In our case the possibilities of sudden death, syncope, heart failure, arrhythmia, and malignant tumor all demanded prompt surgical treatment, and the resected specimen provided the diagnosis. The prognosis of benign cardiac tumor depends on resectability. After complete resection the prognosis is excellent, and adjuvant therapy is not needed. Among reported cases of cardiac neurilemoma, only 3 have required cardiopulmonary bypass [3–5]. Cardiopulmonary bypass provides a stable hemodynamic state and satisfactory visualization to excise the tumor. In our case cardiopulmonary bypass facilitated complete resection of the tumor from the heart and great vessels, particularly the SVC.
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References
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- Forbes A.D., Schmidt R.A., Wood D.E., Cochran R.P., Munkenbeck F., Verrier E.D. Schwannoma of the left atrium: diagnostic evaluation and surgical resection. Ann Thorac Surg 1994;57:743-746.[Abstract]
- Monroe B., Federman M., Balogh K. Cardiac neurilemoma. Report of a case with electron microscopic examination. Arch Pathol Lab Med 1984;108:300-304.[Medline]
- Betancourt B., Defendini E.A., Johnson C., et al. Severe right ventricular outflow tract obstruction caused by an intracavitary cardiac neurilemoma. Successful surgical removal and postoperative diagnosis. Chest 1979;75:522-524.[Abstract/Free Full Text]
- Hallman G.L., Cooley D.A., Webb J.A. Primary tumors of the heart: results of surgical treatment in ten patients. J Cardiovasc Surg 1966;7:447-457.[Medline]
- Cayten C.G., Kastor J.A., Park C.D. Unusual presentation of intrathoracic malignant schwannoma. J Thorac Cardiovasc Surg 1973;66:601-606.[Medline]
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Abstract
Introduction
