Ann Thorac Surg 2007;83:1881-1882
© 2007 The Society of Thoracic Surgeons
Case Reports
Successful Resection of a Primary Left Ventricular Schwannoma
Saverio La Francesca, MD,
Igor D. Gregoric, MD,
William E. Cohn, MD,
O.H. Frazier, MD*
Department of Cardiovascular Surgery, Texas Heart Institute at St. Luke’s Episcopal Hospital, Houston, Texas
Accepted for publication December 11, 2006.
* Address correspondence to Dr Frazier, Texas Heart Institute, PO Box 20345, MC3-147, Houston, TX 77225–0345 (Email: lschwenke{at}heart.thi.tmc.edu).
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Abstract
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Nerve sheath neoplasm of the heart is rare. We report the case of a patient with a giant schwannoma of unique ventricular origin. Resection of the schwannoma and subsequent coronary reconstruction were required.
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Introduction
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Nerve sheath neoplasm of the heart is among the rarest of the primary neurogenic tumors [1]. We report the case of a giant schwannoma of unique ventricular origin that required coronary reconstruction.
A 30-year-old asymptomatic woman came to our institution because of a chest-wall mass on the left side of her sternum. An echocardiogram and subsequent computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a large (4 cm x 4 cm x 9 cm) multilobed cardiac mass involving the anterior and lateral surface of the superior half of the left ventricle and wrapping posterior to the pulmonary artery and ascending aorta. Left ventricular function was normal.
Coronary angiography revealed that the proximal left anterior descending (LAD) and circumflex arteries ran through the mass and were elevated from the surface of the heart (Fig 1). Histologic analysis of an open heart biopsy specimen showed spindle cells with round or elongated nuclei surrounded by fibrillary acidic protein. Immunohistochemistry results revealed the cells were positive for S-100 protein and negative for cytokeratin, CD34+ cells, desmin, and calretinin.
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Fig 1. Coronary angiogram is presented with approximate outlines (superimposed) of the left ventricle and giant left ventricular schwannoma. One portion of the left anterior descending artery (arrow) and the distal left main and proximal circumflex coronary arteries were completely surrounded by tumor and were resected.
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The proximal halves of the LAD and circumflex were found to be deep within the mass, in fissures between the lobes. After institution of cardiopulmonary bypass (CPB), a tissue plane was identified adjacent to the inferior aspect of the mass that allowed the lobules to be carefully shelled off of the myocardium; however, numerous septal branches of the proximal LAD dove through the tumor and had to be divided. At the confluence of the LAD and circumflex arteries, the epicardial coronary arteries were circumferentially invested in the tumor, requiring division and resection of the left main artery. The tumor was completely resected (Fig 2).
Two segments of saphenous vein graft were used as bypasses from the ascending aorta to the divided arteries in an end-to-end fashion. An intraaortic balloon pump was inserted to facilitate weaning from CPB.
Cardiogenic shock developed 12 hours postoperatively. Transesophageal echocardiography (TEE) showed septal dyskinesis. The patient was returned to the operating room for exploratory surgery. Clots were found in the grafts to the LAD and obtuse marginal arteries. A thrombectomy was performed, and the grafts were revised and reconnected to improve the runoff. After blood flow was reestablished, the myocardium remained stunned, and a CentriMag left ventricular assist device (LVAD) (Levitronix LLC, Waltham, MA) was inserted. Heparin was administered for anticoagulation. After 48 hours, the patient was weaned from the device.
The patient was discharged home on postoperative day 28. TEE showed severe septal-basal hypokinesis and an ejection fraction of 0.35.
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Comment
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Few cases of primary cardiac schwannomas have been reported [2–5]. In our patient, the tumor originated from Schwann cells within the left ventricle. Because clinical differentiation of cardiac schwannomas from other cardiac neoplasms is virtually impossible, a biopsy was performed to confirm the diagnosis.
The location of the tumor and the inclusion of the left main and part of the anterior descending coronary arteries necessitated coronary reconstruction. The acute loss of several septal perforators from the proximal LAD probably resulted in transient cardiac compromise. In addition, the patient’s cancer probably contributed to her hypercoaguable state, resulting in the occluded bypass grafts. This further caused the deterioration of her cardiac function, which did not immediately improve after blood flow was restored to the coronary arteries. Therefore, temporary LVAD support was initiated, and anticoagulants were administered. After 48 hours, the patient’s cardiac function improved enough that LVAD support could be discontinued. Coumadin (Bristol-Myers Squibb, Princeton, NJ) was substituted for heparin for the patient’s long-term anticoagulation regimen.
The goal of surgical therapy is complete, margin-free resection and necessary reconstruction, which in this case required coronary artery bypass grafting. Schwannoma, because of its tendency to push surrounding structures aside rather than infiltrate them, may in fact be resectable even when extensive involvement suggests resection would be difficult, if not impossible.
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References
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- Factor S, Turi G, Biempica L. Primary cardiac neurilemoma Cancer 1976;37:883-890.[Medline]
- Morishita T, Yamazaki J, Ohsawa H, et al. Malignant schwannoma of the heart Clin Cardiol 1988;11:126-130.[Medline]
- Jassal DS, Legare JF, Cummings B, et al. Primary cardiac ancient schwannoma J Thorac Cardiovasc Surg 2003;125:733-735.[Free Full Text]
- Nakamura K, Onitsuka T, Yano M, Yano Y. Surgical resection of right atrial neurilemoma extending to pulmonary vein Eur J Cardiothorac Surg 2003;24:840-842.[Abstract/Free Full Text]
- Bottio T, Gerosa G. Clinical-pathologic conference in cardiac surgery: malignant schwannoma of the heart J Thorac Cardiovasc Surg 2005;130:202-205.[Free Full Text]
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Abstract
Introduction
