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J Thorac Cardiovasc Surg 2006;131:224-226
© 2006 The American Association for Thoracic Surgery

Brief Communication

Leiomyosarcoma of the left atrium mimicking a left atrial myxoma

Alessandro Mazzola, MD a , Jean-Philippe Spano, MD, PhD b , Marialuisa Valente, MD c , Renato Gregorini, MD a , Carmine Villani, MD a , Mauro Di Eusanio, MD a , Marco Ciocca, MD a , Ugo Minuti, MD a , Raffaele Giancola, MD a , Cristina Basso, MD, PhD c , Gaetano Thiene, MD, FRCP c , *

a Department of Cardiac Surgery, G. Mazzini Hospital, Teramo, Italy
b Département d'Oncologie Médicale, Pitié-Salpétirère, Paris, France
c Institute of Pathological Anatomy, University of Padua Medical School, Padua, Italy

Received for publication June 15, 2004; revisions received July 1, 2005; accepted for publication July 19, 2005.

* Address for reprints: Gaetano Thiene, MD, FRCP, Institute of Pathological Anatomy, Via A. Gabelli, 61, Padua 35121, Italy (Email: gaetano.thiene{at}unipd.it).

Unlike benign neoplasms, primary cardiac malignancies are rarely intracavitary and, when located in the left atrium, may mimic myxoma on cardiac imaging. Cardiac surgeons should be alerted to ask for prompt surgical pathologic study in any case of intracavitary cardiac mass.

Clinical Summary

A 21-year-old woman was admitted to the hospital with pulmonary edema and shock. Transthoracic echocardiography showed a large (4 x 7-cm) mobile mass within the left atrium obstructing the mitral valve orifice during diastole (Figure 1). The diagnosis of left atrial myxoma was put forward, and an emergency operation was performed with the patient on cardiopulmonary bypass. The left atrium was approached via an extended transseptal incision. The left atrium was occupied by a whitish mass, and the tumor appeared entrapped in the mitral orifice without infiltration of the leaflets or the annulus. The mass originated from the roof of the left atrium, between the pulmonary veins. The tumor and the underlying endocardium were smoothly dissected from the myocardium of the atrial wall, which appeared soft and without evidence of invasion. The operation was performed without complications.


Figure 1
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  		Figure 1. Preoperative transthoracic echocardiography. A, A mass is visible occupying the left atrial cavity during systole. B, The mass is moving downward during diastole to occupy the mitral valve orifice.

 
At gross examination, the mass appeared whitish and ovoid. It measured 7 x 3 x 3.5 cm and weighed 51 g. The surface was slightly irregular and wrinked, hard to cut with a knife. Accessory clusters of small round masses were also present. Microscopic examination of histologic sections revealed a storiform proliferation with pleomorphic cells enmeshed in a myxoid extracellular matrix with a high mitotic index (Figure 2, A and B). Necrotic foci and lymphocytic-plasmacellular infiltrates were also observed. At immunohistochemistry, the neoplastic cells appeared vimentin, desmin, and {alpha}-smooth muscle actin positive (Figure 2, C and D) and myogenin negative, in keeping with a pleomorphic leiomyosarcoma.


Figure 2
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  		Figure 2. Surgical pathologic investigation of the resected mass. A and B, At histologic examination, storiform proliferation of pleomorphic cells with frequent mitoses was seen (stain, hematoxylin and eosin; original magnification, 15x and 160x, respectively). C and D, Positive immunohistochemistry findings for {alpha}-smooth muscle cell and desmin (original magnification, 32x).

 
The patient was treated with anthracycline-based chemotherapy (doxorubicin plus fosfamide plus cisplatin alternated with fosfamide plus cisplatin every 15 days for 2 months). Eleven months later, the patient underwent reoperation for suspected recurrence of the tumor on the anterior mediastinum, but the removed mass turned out to be a reactive hyperplasia of the thymus secondary to chemotherapy. At 24 months from surgical resection, the patient is well, without evidence of local recurrence or metastases on computed axial tomography.

Discussion

Primary leiomyosarcoma of the left atrium is extremely rare and has a poor prognosis. Symptoms of left atrial leiomyosarcoma are generally related to obstruction of the left atrial cavity, pulmonary veins, or mitral valve orifice. 1,2 Go The extension of the tumor to the pulmonary veins often makes pneumonectomy necessary to obtain radical resection. 3 Go

In our case, the clinical presentation and the echocardiographic features strongly suggested a left atrial myxoma, but the rough surface and the unusual origin from the roof of the left atrium argued against a benign tumor. Thorough histologic and immunohistochemical investigations allowed to disclose the malignant features of leiomyosarcoma. As in the case reported by Evans and Haw, 3 Go the tumor was attached to the endocardial surface without infiltration of the atrial wall, thus making its complete removal feasible. Although we believed that the tumor was removed completely and, in the early postoperative period, no metastases could be demonstrated, we decided to treat the patient with anthracycline-based chemotherapy to destroy or prevent repetitions. As reported by Pessotto and associates, 4 Go our case supports the hypothesis that multimodal treatment of cardiac leiomyosarcomas may prolong survival with a good quality of life. 1 Go

From this experience, we have learned that it may be difficult to distinguish a noninfiltrating intracavitary malignancy from a left atrial myxoma by using echocardiography. Skilled histopathologic examination is mandatory in any apparently benign cardiac mass to exclude malignancy 5 Go and, if so, to allow early chemotherapic therapy, which can be successful, as in this case.

References

  1. Babatasi G, Massetti M, Galateau F, Khayat A. Leiomyosarcoma of the pulmonary veins extending into the left atrium or left atrial leiomyosarcoma. multimodality therapy. J Thorac Cardiovasc Surg 1998;4:665-667.
  2. Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac leiomyosarcomas. Ann Thorac Surg 1991;51:999-1001.[Abstract/Free Full Text]
  3. Evans JE, Haw MP. Surgical clearance of invasive cardiac leiomyosarcoma with concomitant pneumonectomy. Eur J Cardiothorac Surg 2003;24:843-846.[Abstract/Free Full Text]
  4. Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, et al. Primary cardiac leiomyosarcoma. seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol 1997;60:91-94.[Medline]
  5. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg 1997;12:730-737.[Abstract/Free Full Text]



This article has been cited by other articles:


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 ESC Textbook of Cardiovascular MedicineHome page
G. Thiene, M. Valente, M. Lombardi, and C. Basso
CHAPTER 20 Tumours of the Heart
ESC Textbook of Cardiovascular Medicine, January 1, 2009; 2(1): med-9780199566990-chapter - med-9780199566990-chapter.
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