<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_raon-2016-0051_s_007_w2aab3b8c70b1b7b1aab1c17b1Aa"><h3>Background</h3><p>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.</p></sec><sec id="j_raon-2016-0051_s_008_w2aab3b8c70b1b7b1aab1c17b2Aa"><h3>Methods</h3><p>Cases from the literature based on PubMed search and a case from our institution were included.</p></sec><sec id="j_raon-2016-0051_s_009_w2aab3b8c70b1b7b1aab1c17b3Aa"><h3>Results</h3><p>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.</p></sec><sec id="j_raon-2016-0051_s_010_w2aab3b8c70b1b7b1aab1c17b4Aa"><h3>Conclusions</h3><p>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.</p></sec></abstract>

BackgroundLeiomyosarcoma 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.MethodsCases from the literature based on PubMed search and a case from our institution were included.ResultsSixty-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.ConclusionsBased 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.

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

Radiology and Oncology

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

BackgroundLeiomyosarcoma is a rare malignant mesenchymal tumour. Some cases of leiomyosarcoma of the renal vein (LRV) have been reported in the literature, but...

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

Article Category: Research Article

Pubblicato online: 23 dic 2016

Pagine: 56 - 64

Ricevuto: 11 giu 2016

Accettato: 05 ott 2016

DOI: https://doi.org/10.1515/raon-2016-0051

Parole chiave
leiomyosarcoma, renal vein, surgery, outcome

© 2017 Marko Novak, Andraz Perhavec, Katherine E. Maturen, Snezana Pavlovic Djokic, Simona Jereb, Darja Erzen

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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

Article Category: Research Article

Pubblicato online: 23 dic 2016

Pagine: 56 - 64

Ricevuto: 11 giu 2016

Accettato: 05 ott 2016

DOI: https://doi.org/10.1515/raon-2016-0051

Parole chiaveleiomyosarcoma, renal vein, surgery, outcome

© 2017 Marko Novak, Andraz Perhavec, Katherine E. Maturen, Snezana Pavlovic Djokic, Simona Jereb, Darja Erzen

This article is distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Parole chiave
leiomyosarcoma, renal vein, surgery, outcome