In this paper, the authors report the case of a 28-year-old man with pulmonary vein leiomyosarcoma presenting subacute respiratory distress. Thoracic computed tomography and transoesophagal ultrasonographic examination of the heart suggested the diagnosis of a heart tumour revealed by the obstruction of the mitral valve and pulmonary oedema. Emergency cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the right inferior pulmonary vein and extending into the left atrium. The clinical evolution was complicated because of a sudden local relapse. The patient underwent a second cardiac intervention involving lower right lobectomy followed by adjuvant chemotherapy with an ifosfamide-adriamycin combination. This treatment failed to control the disease and a third cardiac intervention was necessary with second-line gemcitabine-paclitaxel adjuvant chemotherapy. Further recurrences were observed with metastases first in the scalp and then in the spine and adrenal glands leading to the death of the patient 2 years after the diagnosis.
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