Eur J Cardiothorac Surg 2003;24:840-842
© 2003 Elsevier Science NL
Surgical resection of right atrial neurilemoma extending to pulmonary vein
Kunihide Nakamura*,
Toshio Onitsuka,
Mitsuhiro Yano,
Yoshikazu Yano
Department of Surgery 2, Miyazaki Medical College, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Received 10 April 2003;
received in revised form 17 June 2003;
accepted 18 June 2003.
* Corresponding author. Tel.: +81-985-852291; fax: +81-985-855563
e-mail: shiori{at}post1.miyazaki-med.ac.jp
 |
Abstract
|
|---|
An asymptomatic cardiac neurilemoma was found in a 33-year-old woman. The tumor was on the anterior surface of the right atrium extending to the right upper pulmonary vein across the interatrial groove. The tumor was resected completely and right atrium, interatrial septum, left atrium and right superior pulmonary vein were reconstructed with cardiopulmonary bypass. The patient recovered uneventfully and had no recurrence 1 year after the operation. We reviewed ten reported cases of cardiac neurilemoma, including our patient.
Key Words: Cardiac neurilemoma • Surgery • Right atrium
|
1. Introduction
|
|---|
Primary cardiac tumor remains rare and the incidence was between 0.001 and 0.28% in reported or collected autopsy series [1]. The majority of primary cardiac tumors are benign and about half of these are myxoma. Lipoma, papillary fibroelastoma and rhabdomyoma were reported in decreasing order of frequency [1]. Primary cardiac neurilemoma is extremely rare indeed and only nine cases including two autopsy cases have been reported in the world literature (Appendix A) [2–10].
|
2. Case report
|
|---|
A 33-year-old woman complained of cough and sputum and was admitted to a family doctor. Although the patient's symptoms resolved with medical treatment, her chest X-ray films showed abnormal cardiac silhouette and echocardiographic examination revealed a right atrial mass. She was referred to our hospital for further examination being suspicious of cardiac tumor. Occupational and family history was normal. She had no heart murmur and breath sounds were clear. The ECG showed a normal sinus rhythm with no axis deviation. Chest X-ray films revealed bulging of right lower cardiac border with a cardiothoracic ratio of 47%. Transthoracic and transesophageal echocardiography revealed a 5-cm mass in the right atrium with no pericardial effusion. Computed tomography confirmed that the location of the mass was under the epicardium and it severely displaced the right atrium and right lateral wall of the left atrium. Magnetic resonance imaging (MRI) showed that the tumor was high signal intensity on T2-weighted images and intensity of T1-weighted images resembled that of myocardium (Fig. 1a)
. Following gadolinium-DTPA, the tumor showed inhomogeneous enhancement (Fig. 1b). These findings of MRI suggested the tumor as a neurogenic tumor or hemorrhage in an angiosarcoma. Cardiac catheterization was normal and coronary angiogram showed a feeding artery originating from the circumflex artery. The patient was planned to undergo complete resection of the tumor under cardiopulmonary bypass (CPB) in anticipation of a malignant tumor.
View larger version (71K):
[in this window]
[in a new window]
|
Fig. 1. (a) Magnetic resonance imaging revealed a 5-cm tumor on the right atrium extending to the left atrium. (b) The tumor showed inhomogeneous enhancement on contrast-enhanced magnetic resonance imaging.
|
|
A small median sternotomy was performed, and the pericardium was opened. A spherical tumor was on the anterior surface of the right atrium extending to the right upper pulmonary vein. After the establishment of CPB, the right atrium was opened and an atrial septum was involved in the tumor, so the atrial septum was opened and the tumor was resected completely together with a rim of septum, left atrium, and pulmonary vein around its base. The defect of pulmonary vein and the left atrium were replaced with an autologous pericardium and the atrial septum and right atrial wall was reconstructed by equine pericardial patch (Xenomedica, Edwards Lifescience LCC, Irvine, CA, USA). The tumor was weighed 65 g and microscopic sections showed fascicular proliferation of spindle-shape type with nuclear palisading (Fig. 2)
. The immunohistochemical study was compatible with that of a neurilemoma (positive for s-100 and negative for actin). The patient recovered uneventfully and there was no evidence of recurrence 1 year after the operation.
View larger version (106K):
[in this window]
[in a new window]
|
Fig. 2. Microscopic findings. This section of the tumor shows fascicular proliferation of spindle-shape tumor cells with nuclear palisading (H&E stain, original magnification x120).
|
|
|
3. Discussion
|
|---|
Cardiac branches of the vagus nerve and cardiac plexus are suspected of being an origin of the primary cardiac neurilemoma [3,5]. The location of the tumor supports this conception because seven cases arose in the right side of the heart [2–5,8–10] and only two in the left [6,7]. Primary cardiac tumors are predominant in the left atrium and the majority of those were benign compared with about half of the lesions identified in the right atrium. Therefore, identification of a right atrial mass is much likely to presage eventual identification of a malignant etiology than a left atrium. However, some tumors that arise in the right side of the heart near the cardiac plexus may be neurogenic tumor.
Six out of ten patients of cardiac neurilemoma including our case were female and shows a similar ratio in proportion to the number of female patients with other primary cardiac tumors. The average age of ten patients ranging from 12 to 72 years old was 43 years old, and the average age of the operative cases was 38 years old. Primary cardiac tumors are encountered in every age group and Purkinje cell tumors, rhabdomyomas, and fibroma mainly occur in children. Judging from ten cases, it is unlikely that cardiac neurilemoma tends to occur in children. Five patients were symptomatic in the seven operated patients [2,5,7,8,10]. Four patients had exertional dyspnea or shortness of breath [2,5,8,10], three had chest pain [5,7,10], and one had easy fatigability [2]. Cardiac enlargement or a bizarre cardiac contour on chest X-ray turned out to be a clue to reveal an asymptomatic cardiac neurilemoma in one patient [9]. In the course of unrelated cardiac operation, right atrial cardiac neurilemoma was found incidentally and resected in one patient [3]. In patients with cardiac tumors, the history and clinical findings primarily depend on the anatomic location of the tumor rather than the histopathology, and tumors manifest symptomatology and clinical signs by virtue of tumor growth that impedes cardiac hemodynamics.
Patients with benign cardiac tumors have excellent prognosis after complete resection. Excepting two autopsies cases [4,6], all but one patient [2] had their surgical procedures for curative resection performed with CPB, and their prognoses were good with no recurrence including one exceptional operative case without CPB. As a surgical approach is warranted and a precise diagnosis is made by histopathological study after the operation, the tumor should be removed with a negative surgical margin. In our case, the tumor was found originating from the anterior right atrium extending to the right superior pulmonary vein and it was resected from the right atrium, interatrial septum, left atrium, and superior pulmonary vein by incising normal structure about 5 mm outside the tumor.
Clinical diagnostic methods for heart disease have progressed remarkably and the incidence of cardiac tumor may be increasing. Prompt surgical resection is mandatory with the diagnosis of cardiac tumor, and extensive surgical resection of cardiac tumor with reconstruction of the myocardial chamber is required.
|
appendix a: summary of reported cases of cardiac neurilemoma
|
|---|
Appendix A
|
References
|
|---|
- McAllister H.G., Jr Primary tumor and cysts of the hear and pericardium. Current problems in cardiology. Chicago, IL: Year Book Medical, 1979.
- 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;6:447-457.
- Gleason T.H., Dillard D.H., Gould V.E. Cardiac neurilemoma. NY State J Med 1972;72:2435-2436.[Medline]
- Factor S., Turi G., Biempica L. Primary cardiac neurilemoma. Cancer 1976;37:883-890.[Medline]
- Betancourt B., Defendini E.A., Johnson C., De Jesus M., Villamil A.P., Cruz A.D., Medina J.C. Severe right ventricular outflow tract obstruction caused by an intracavitary cardiac neurilemoma. Chest 1979;75:522-524.[Abstract/Free Full Text]
- 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]
- 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]
- Kodama M., Aoki M., Sakai K. Primary cardiac neurilemoma. Circulation 1995;92:274-275.[Free Full Text]
- Hashimoto T., Eguchi S., Nakayama T., Ohzeki H., Hayashi J. Successful removal of massive cardiac neurilemoma with cardiopulmonary bypass. Ann Thorac Surg 1998;66:533-535.
- Bizzarri F., Mondillo S., Tanganelli P., Lisi G., Guerrini F., Ammaturo T., Barbati R., Maccherini M., Toscano M. A primary intracavitary right atrial neurilemoma. J Cardiovasc Surg 2001;42:777-779.[Medline]
Top
Abstract
1. Introduction
