Leiomyosarcoma of the renal vein: case report and review of the literature. World wide case series of 67 patients
Background. The purpose of this retrospective study was to better describe the leiomyosarcoma of the renal vein (LRV), to analyse overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS) in LRV world case series and possibly to identify significant predictors of OS, LRFS and DMFS.
Methods. Cases from the literature and case from our institution were included.
Results. Sixty-seven patients with a mean age of 56.6 years were identified; 76.1% were women. Mean tumor size was 8.9 cm; in 68.7% located on the left side. Tumor thrombus extended into the inferior vena cava lumen in 13.4%. All patients but one underwent surgery (98.5%). After a median follow up of 24 months, the OS was 79.5%. LRFS was 83.5% after a median follow up of 21.5 months and DMFS was 76.1% after a median follow up of 22 months. Factors predictive of OS in univariate analysis were surgical margins, while factors predictive of LRFS were inferior vena cava luminal extension and grade. No factors predictive of DMFS were identified. In multivariate analysis none of the factors were predictive of OS, LRFS and DMFS.
Conclusion. LRV is usually located in the hilum of the kidney. It should be considerated in differential diagnosis of renal and retroperitoneal masses, particularly if in women over the age 40, on the left side and in the absence of hematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma multidisciplinary team. LRV should be surgically removed, with negative margins.
KEYWORDS: Leiomyosarcoma, Renal vein, Surgery, Outcome