Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy
Introduction
Primary malignant cardiac tumors occur extremely rarely and are mostly represented by sarcomas [1]. Among these, leiomyosarcomas are exceptional and, excluding those forms involving the pulmonary artery, only 14 surgically treated cases have been extensively described 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14. Radical surgical interventions seem to offer the best palliation, while the role of adjunctive therapies remains controversial 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14. We describe a case of left atrial leiomyosarcoma in which two aggressive surgical interventions followed by adjuvant radiation and chemotherapy offered the longest reported survival with good interim quality of life.
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Case report
A 24-year-old Caucasian male was admitted at a local hospital in March 1988 for a syncope associated with rapid atrial fibrillation. History revealed exertional dysrhythmias of 3 months' duration. After spontaneous conversion to sinus rhythm physical examination as well as ECG and chest X-ray were unremarkable. A 2-D echocardiogram revealed a 5×4.5 cm left atrial (LA) mass arising from the interatrial septum, occuping the atrial cavity and prolapsing through the mitral orifice (Fig. 1). With
Discussion
Primary cardiac neoplasms are rare with an incidence, in autopsy series, ranging from 0.0017 to 0.033%. One fourth of all primary cardiac tumors are malignant and almost all of them are sarcomas [15]. Leiomyosarcomas are even more uncommon with less than 30 cases reported in the literature. Only 14 of them have been treated surgically 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14. In 70% of the operated cases, the tumor originates from the left-sided cardiac chambers, particularly the left atrium,
References (15)
- Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992; 69:...
- Birmann L, Eisenmann B, Rey G, Braganti G, Kieny R, Sacrez A. Leiomyosarcome du coeur gauche à symptomatologie mitrale....
- McAllister HA Jr, Fenoglio JJ. Tumors of the cardiovascular system. In: Atlas of tumor pathology, fasc. 15, second...
- Donovan VM, Summer W, Hutchins GM. Left atrial leiomyosarcoma. Manifestation as unexplained pulmonary vascular disease....
- Reece IJ, Cooley DA, Frazier OH, Hallman GL, Powers PL, Montero CG. Cardiac tumors. J Thorac Cardiovasc Surg 1984; 88:...
- von Segesser L, Cox J, Gross J, Lerch R, Gabathuler J, Glassey F, Gisselbaek A, Faidutti B. Surgery in primary...
- Talley JD, Franch RH, Clements SD, Murphy DA, Sewell CW. Primary right ventricular leiomyosarcoma producing outflow...