HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Massimo Massetti Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Babatasi, G. Articles by Khayat, A. Search for Related Content PubMed PubMed Citation Articles by Babatasi, G. Articles by Khayat, A.

J Thorac Cardiovasc Surg 1998;116:665-666
© 1998 Mosby, Inc.

LETTERS TO THE EDITOR

Leiomyosarcoma of the pulmonary veins extending into the left atrium or left atrial leiomyosarcoma: Multimodality therapy

Caen, France and Washington, DC

Department of Cardiovascular Pathology, CHU Caen and Armed Forces Institute of Pathology (AFIP), Washington, DC^b

To the Editor:

We read with interest the article by Shimono and associates (J Thorac Cardiovasc Surg 1998;115:469-1) titled "Pulmonary Leiomyosarcoma Extending Into Left Atrium or Pulmonary Trunk: Complete Resection With Cardiopulmonary Bypass." Although it is true that primary pulmonary leiomyosarcoma is a rare tumor, we think that the second case reported consisted in a left atrial leiomyosarcoma originating in the atrial wall and extending into the pulmonary veins. The most important series in the world concerning this tumor is the experience of the Armed Forces Institute of Pathology (AFIP), ¹ with 12 cases reported and 6 cases surgically resected. Controversy still exists concerning their origin. The AFIP clearly demonstrated that the majority of cardiac leiomyosarcomas arise in the left atrium; at surgery, some of these appear to arise from a pulmonary vein. For these reasons, the cell origin of cardiac leiomyosarcoma may reside in the smooth muscle media of pulmonary veins. However, it is likely that some leiomyosarcomas originate within the left atrium itself, because the left atrium is a preferred site of origin of most cardiac sarcomas, and because the subendocardial lining of the atrium normally contains bundles of smooth muscle cells. Their large size at the time of diagnosis makes assessment of the precise site of origin impossible in most cases. To the best of our knowledge, 20 cases of left atrial leiomyosarcomas have been previously reported in the literature (Table I). The mean postoperative survival in those patients is 6.8 months. ^1,2 In our opinion, 7 months is insufficient follow-up time to evaluate whether surgery without adjuvant therapy has been successful. Shimono and associates stated that their case was the first report of the resection of a left atrial tumor by thoracotomy through the rib bed, and we understand why the majority of cardiac surgeons relating their experience preferred the sternotomy approach. Surgery is indicated to obtain a definitive histologic diagnosis and to evaluate the prognosis, and as much of the tumor as possible must be removed during the operation. Visualization of tumors in the left atrium appears to be more difficult, and detection of unresected portions of tumor after the operation can be extremely difficult if not impossible. ³ This, evidently, can be extremely difficult in tumors of the left atrium with involvement of the pulmonary veins ⁴ by the thoracotomy approach.

Regarding adjuvant chemotherapy, which was not indicated in the 2 cases reported by Shimono and associates, recent reports seemed to demonstrate the benefit of a chemotherapy regimen in those type of tumors. ^4-6 Doxorubicin-based postoperative adjuvant therapy has been used on the basis of its effectiveness in treating soft tissue sarcomas in other locations. ⁶ Because of the poor prognosis of patients with leiomyosarcomas of the heart and the great vessels, the recommended therapy is surgery followed by chemotherapy and radiation. ^2,4 Some authors ^4,5 have demonstrated that radiotherapy and/or chemotherapy administered in the postoperative period was followed by a significantly prolonged survival time (P = .023). Pulmonary metastases are present in up to 60% ⁴ of patients. Distal microembolization is common; therefore adjuvant therapy, even in the absence of pulmonary nodules, would seem appropriate. In conclusion, total surgical resection (which was best performed with the aid of cardiopulmonary bypass through a median sternotomy), with the addition of chemotherapy, radiotherapy, or both, should offer these patients significant palliation and an opportunity for increased length of survival.

References

1. Burke A, Virmani R. Tumors of the heart and great vessels. Vol 16. Atlas of tumor pathology. Washington: Armed Forces Institute of Pathology; 1996. p. 127-69.
   
2. Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac leiomyosarcomas. Ann Thorac Surg 1991;51:999-1001. [Abstract]
   
3. Fyfe AI, Huckell VF, Burr LH, Stonier PM. Leiomyosarcoma of the left atrium: case report and review of the literature. Can J Cardiol 1991;7:192-6.
   
4. Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of 6 cases. Ann Thorac Surg 1995;59:1487-90. [Abstract/Free Full Text]
   
5. Kruger I, Borowski, A, Horst M, De Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990;38:91-5. [Medline]
   
6. Han P, Drachtman RA, Amenta P, Ettinger LJ. Successful treatment of a primary cardiac leiomyosarcoma with Ifosfamide and Etoposide. J Pediatr Hematol Oncol 1996;18:314-7. [Medline]
   

This article has been cited by other articles:

				M. Malyshev, A. Safuanov, I. Gladyshev, V. Trushyna, L. Abramovskaya, and A. Malyshev Primary Left Atrial Leiomyosarcoma: Literature Review and Lessons of a Case Asian Cardiovasc Thorac Ann, October 1, 2006; 14(5): 435 - 440. [Abstract] [Full Text] [PDF]

This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Massimo Massetti Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Babatasi, G. Articles by Khayat, A. Search for Related Content PubMed PubMed Citation Articles by Babatasi, G. Articles by Khayat, A.