Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy

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Abstract

Primary cardiac sarcomas constitute a rare entity that have been uniformly associated with poor long-term survival. A case of left atrial leiomyosarcoma involving the interatrial septum and the right atrial free wall and presenting with syncope and atrial fibrillation, is described. Two extensive surgical excisions followed by adjuvant radiation and chemotherapy improved survival with a good quality of life. This approach of combined surgical, medical and radiation therapy may offer better longterm outcome, since our patient is the longest survivor thus far reported.

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.

Section snippets

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,

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