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Primary cardiac leiomyosarcoma is a very rare condition. Only 15 cases have been reported in the literature. We report the case of a 27 year old man admitted to hospital for chest pain in March 1985. Echocardiography showed a right ventricular tumour which was completely resected at surgery. The diagnosis of leiomyosarcoma was confirmed by histological examination. After 22 months follow-up, the patient was still alive despite pulmonary metastases. The diagnosis of cardiac tumour used to be made post-mortem but since the introduction of new methods of investigation, especially 2D echocardiography, the diagnosis can be made at an early stage and allows rapid surgical resection, the only means of obtaining the histological diagnosis and of completely curing benign tumours.
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