Leiomyosarcoma of the renal vein. Case report.

The authors report a new case of leiomyosarcoma of the right renal vein, ma rked by a rapidly fatal course within 9 months, despite the initial absence of metastasis. This exceptional vascular tumour (only about thirty cases have been publish ed) must be distinguished from primary renal leiomyosarcoma and retroperito neal leiomyosarcomas involving adjacent structures. This difficult diagnosi s was suggested by preoperative CT and angiography. Leiomyosarcomas of the renal vein generally have an unfavourable outcome in the medium term. The p rognosis is related to the localized nature of the tumour and the risk of l ocal and distant recurrence. Limited tumour resection is rarely. sufficient and radical nephrectomy is usually necessary, possibly associated with a v ascular procedure on the inferior vena cava in the case of contiguous exten sion.