The authors report the case of a 42-year-old woman in whom a tumour of the inferior vena cava was discovered intraoperatively. Histological examination of the specimen confirmed the diagnosis of leiomyosarcoma. Malignant tumours of the inferior vena cava are very rare and about 95% of these tumours are leiomyosarcomas. These tumours are diagnosed preoperatively in only 10% of cases. CT and MRI allow a precise staging assessment. Radical surgery and a combination of radiotherapy-chemotherapy constitute optimal treatment for leiomyosarcoma of the inferior vena cava, ensuring long survival, but recurrences are frequent.
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