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. 2016 Dec 23;51(1):56-64.
doi: 10.1515/raon-2016-0051. eCollection 2017 Mar 1.

Leiomyosarcoma of the renal vein: analysis of outcome and prognostic factors in the world case series of 67 patients

Marko Novak  1 , Andraz Perhavec  1 , Katherine E Maturen  2 , Snezana Pavlovic Djokic  3 , Simona Jereb  4 , Darja Erzen  1
Affiliations

Leiomyosarcoma of the renal vein: analysis of outcome and prognostic factors in the world case series of 67 patients

Marko Novak et al. Radiol Oncol. .

Abstract

Background: Leiomyosarcoma is a rare malignant mesenchymal tumour. Some cases of leiomyosarcoma of the renal vein (LRV) have been reported in the literature, but no analysis of data and search for prognostic factors have been done so far. The aim of this review was to describe the LRV, to analyse overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS) in LRV world case series and to identify significant predictors of OS, LRFS and DMFS.

Methods: Cases from the literature based on PubMed search and a 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 tumour size was 8.9 cm; in 68.7% located on the left side. Tumour 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.

Conclusions: Based on the literature review and presented case some conclusions can be made. LRV is usually located in the hilum of the kidney. It should be considered in differential diagnosis of renal and retroperitoneal masses, particularly in women over the age 40, on the left side and in the absence of haematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma multidisciplinary team. LRV should be surgically removed, with negative margins.

Keywords: leiomyosarcoma; outcome; renal vein; surgery.

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Figures

Figure 1
Figure 1
Flow chart of identification, screening, eligibility and inclusion of studies.
Figure 2
Figure 2
Enhanced computed tomography showing retroperitoneal tumour, interposed between the aorta and the left kidney, axial (A) and coronal plane (B). Separately removed satellite node in coronal plane, arrow (C).
Figure 3
Figure 3
(A) A gross specimen of renal vein leiomyosarcoma. The tumour is well-circumscribed, is lying in the renal hilum, without infiltration of the renal parencyma. (B) Hematoxylin & Eosin stain section. Showing the vascular lumen (L) and the tumour (TU) growing from the wall of the renal vein (WV). Immunohistochemical stains for SMA (C) and desmin (D) showing strong positivity.
Figure 4
Figure 4
Clinical presentation of leiomyosarcoma of the renal vein cases.
Figure 5
Figure 5
Age distribution of leiomyosarcoma of the renal vein patients.
Figure 6
Figure 6
Kaplan-Meier curve of overall freesurvival.
Figure 7
Figure 7
Kaplan-Meier curve of local recurrence free survival.
Figure 8
Figure 8
Kaplan-Meier curve of distant metastases free survival.
See this image and copyright information in PMC

References

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