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Ann Thorac Surg 2005;79:1771-1774
© 2005 The Society of Thoracic Surgeons
a Department of Medicine, Section of Cardiology, Houston, Texas, USA
b Department of Pathology, Baylor College of Medicine, Houston, Texas, USA
c The Methodist Hospital, Houston, Texas, USA
Accepted for publication November 6, 2003.
* Address reprint requests to Dr Nagueh, 6550 Fannin St, SM-1246, Houston, TX, USA 77030-2717
sherifn{at}bcm.tmc.edu
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Introduction |
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A 57-year-old woman presented with dyspnea and was found to have a left atrial tumor by transthoracic echocardiography. She subsequently underwent transesophageal echocardiography (TEE) for further elucidation of origin and tumor size (Figs 1A, 1B, 1C; top panel; TEE views). A TEE showed the tumor originating from the interatrial septum, having deposits along the anterior left atrial wall.
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At surgery, the tumor had a smooth surface (Fig 2; large arrow), was highly vascular, and had indeed originated from the interatrial septum as previously shown by TEE. Histopathology of the lesion revealed a neoplasm of sparse cellularity with a myxoid matrix and focal hypercellular regions with closely packed hyperchromatic cells (Fig 3). Immunohistochemical staining with antibodies against desmins, 
-actin, and muscle specific actin (HHF-35) demonstrated immunoreactivity of the tumor cells, confirming the diagnosis of a leiomyosarcoma. No immunoreactivity was observed with antibodies to keratin, vascular markers (CD31, CD34, factor VIII), hmb-45, S-100, or melan-A.
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Postoperatively the patient received systemic chemotherapy, but eventually died 10 months later from abdominal spread of the tumor without evidence of cardiac recurrence.
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The clinical presentation is frequently one of pulmonary congestion related to the increased left atrial pressure [2]. The tumor can do this through several mechanisms, including obstruction of the mitral valve, which results in mitral stenosis, as occurred in this case in which the patient had a diastolic transmitral pressure gradient by Doppler echocardiography of 4 to 8 mm Hg (depending on heart rate). Obstruction of the pulmonary veins [3] can also result in increased pulmonary venous pressure, even though the left atrial pressure may not be markedly increased. Therefore, proper examination of the pulmonary veins by TEE is important, given the need to tackle these lesions from the symptomatology and recurrence perspectives. Finally, the tumor can result in a decrease in left atrial compliance, thereby leading to a higher left atrial pressure for any given volume. In addition, the tumor may cause constitutional symptoms.
Cardiac sarcomas are usually treated with several modalities, but successful and complete surgical resection remains a cornerstone and an important determinant of local recurrence and duration of disease-free survival. There are a number of surgical options, including standard resection, cardiac transplantation, and extracorporeal resection. The complete resection, although the heart is still in place in vivo, is usually hampered by the extensive nature of the tumor and its location, which hinders easy access for surgery. The inadequacy of the in vivo approach of resection of such tumors is supported by previous reports showing early cardiac recurrence after such surgery [2, 4]. This case illustrates the daunting task of a complete surgical resection, with the heart in place, when facing an extensive highly aggressive tumor that originated from a large area of the interatrial septum, which practically filled the whole left atrium. In addition, it was realized through TEE that complete resection in vivo would not be feasible without removing the large tumor deposits in the anterior left atrial wall.
In regard to cardiac transplantation [5], the limited supply of donor hearts, the difficulty of waiting for a suitable donor while a potentially curable case becomes inoperable, along with the potentially deleterious effects of immunosuppressive therapy on patients who may have received chemotherapy (or who may need it in the future) limit the utility of this approach.
For the reasons previously described, a radical surgical approach was taken for our patient in which complete resection of the left atrial sarcoma was achieved by a combination of three techniques: (1) cardiac explantation, (2) extracorporeal resection of the tumor with cardiac reconstruction, and (3) cardiac autotransplantation (cardiac reimplantation). As illustrated in this case, the surgical management was successful in preventing local recurrence. However the patient later succumbed to abdominal metastasis. We believe the outcome of this case and others, in which future metastatic recurrence was documented outside the heart, highlight the need for aggressive systemic therapy for such tumors.
In summary, this case offered a rare opportunity to show the rapid growth of this malignant tumor because the patient waited 1 month before undergoing ex-vivo resection. This also illustrates the successful loco-regional outcome of the radical surgical approach.
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This article has been cited by other articles:
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  J. Canadyova, M. Setina, S. Smetanova, and A. Mokracek Leiomyosarcoma of the Left Atrium Asian Cardiovasc Thorac Ann, February 1, 2008; 16(1): e7 - e9. [Abstract] [Full Text] [PDF]
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 I. M. Keeling, F. Ploner, and B. Rigler Familial cardiac angiosarcoma. Ann. Thorac. Surg., October 1, 2006; 82(4): 1576 - 1576. [Full Text] [PDF]
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M. J. Reardon, S. C. Malaisrie, J.-C. Walkes, A. A. Vaporciyan, D. C. Rice, W. R. Smythe, C. A. DeFelice, and Z. J. Wojciechowski Cardiac Autotransplantation for Primary Cardiac Tumors Ann. Thorac. Surg., August 1, 2006; 82(2): 645 - 650. [Abstract] [Full Text] [PDF]
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