Rapidly Growing and Recurrent Pulmonary Venous Leiomyosarcoma Involving the Left Atrium: Detected by Echocardiography with its Fatal Consequences - A Case Report

Leiomyosarcoma is a rare form of malignant tumour of smooth muscle cells and its course is very unpredictable. One 19-year-old male patient with rapidly growing pulmonary venous leiomyosarcoma was detected by echocardiography in the Fujita Health University Hospital, Japan. Initially he developed exertional dyspnoea, paroxysmal nocturnal cough and haemoptysis. Symptoms were worsening rapidly. Contrast CT revealed left atrial dilatation and a filling defect at the commencement of right upper pulmonary vein and left atrium. Echocardiography showed a large, bilobular, echogenic mass within the left atrium and obstructing the mitral valve annulus during diastole. For maintaining the haemodynamic balance emergency operation was performed. Within very short interval subsequent two operations were performed due to recurrent mass at the same site. After 1^st operation chemotherapy and after 3^rd operation radiotherapy were given but the patient developed metastatic lesions in brain and abdomen. He subsequently underwent operation for focal brain lesion. The histological and immunohistochemical appearance was that of an intimal sarcoma, most closely resembling malignant histiocytoma. Usually 1^st operation is possible in primary leiomyosarcoma and due to rapid metastasis subsequent operations are not possible, but this case was an exceptional one. Enbloc dissection was carried out in three stages, each time histological diagnosis was debatable and finally confirmed as primary pulmonary venous leiomyosarcoma on autopsy specimen.