Cardiac rhabdomyosarcoma of the left atrium

Primary cardiac tumours are quite rare. Only 7 cases were reported in a series of 12,000 autopsies, and its prevalence is less than 0.1% [1]. Malignant tumours account for approximately 15% of primary cardiac tumours, and sarcomas are the most common [2]. Cardiac sarcomas are quite rare and have been published as isolated case reports. Rhabdomyosarcomas account for 20% of overall cardiac sarcomas and are more prevalent in adults. Rhabdomyosarcomas most commonly originate from the ventricles. Echocardiographic (ECHO) findings can provide diagnosis, while electrocardiography and X-ray findings can be nonspecific [2]. In addition to cardiac magnetic resonance imaging (MRI), computed tomography (CT) is used for diagnostic purposes. Primary cardiac sarcomas cause death due to obstruction of blood flow through the heart. Surgical resection is the treatment of choice for all primary cardiac tumours. Additional treatment options include chemotherapy and radiotherapy. In the present report, a case of rhabdomyosarcoma in the left atrium is described.

Posteroanterior (PA) chest radiograph was performed in a 44-year-old female patient with congenital kyphosis, complaining of cough, shortness of breath and weakness. On subsequent ECHO, a 4.3 cm × 2.8 cm left atrial mass was detected beginning from the annulus of the anterior mitral leaflet and extending onto the anterior leaflet. The mass extended towards to the left ventricle inlet during each systole so that 2–3 degrees of mitral insufficiency occurred (Fig. 1A). On 19th January, 2011, the patient was operated on and a tumour was found in the left atrium with extensive infiltration into the atrial septum and adhesion of the anterior leaflet of the mitral valve, which was slightly thickened. The tumour was resected together with part of the atrial septum, and the anterior mitral leaflet was preserved. The atrial septum was reconstructed, and the mitral valve was repaired. A safety margin was confirmed by frozen section technique. Histopathological examination of the surgical specimen revealed pleomorphic sarcoma, while immunohistochemical examination showed negative staining of the neoplastic cells with caldesmon, s-100 and cd-34. Additionally, desmin was focal positive, and smooth muscle actin was positive. The histopathological and the immunohistochemical findings were consistent with rhabdomyosarcoma (Fig. 2). There were no malignant cells on the surgical margins (R0 resection). No systemic...


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