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

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 Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation

“…A recent study of prognostic factors shows that in univariate analysis, factors predictive of overall survival are surgical margins, while factors predictive of local recurrence free survival are IVC luminal extension and grade. No factors predictive of distant metastasis free survival were identified [ 3 ]. It remains difficult to evaluate the true overall survival rate, as most reports do not have long follow-ups.…”

“…They more often arise from the inferior vena cava, far less commonly from the pulmonary artery, and rarely in systemic arteries [ 2 ]. Primary LMS of the renal vein are rare with only 67 cases reported worldwide [ 3 ]. They occur predominantly in females with a peak in the fifth and sixth decades [ 4 , 5 ].…”

Leiomyosarcoma of the inferior vena cava. Our experience and a review of the literature

“…Renal LMSs develop from the renal structures that have smooth muscle layers, such as the renal capsule, pelvis and vein. The most frequent site is the renal vein [29], being also the most common location for extracaval LMSs, followed by renal capsule [30] and renal pelvis [31]. Fifteen percent of all soft tissue sarcomas are located in the retroperitoneum, 20% of them being LMSs.…”

Leiomyosarcoma of the inferior vena cava. Clinical observation

“…Опухоль НПВ следует отличать от лейомиосаркомы, возникающей из окружающих структур, главным образом из почки и забрюшинного пространства. Наиболее частым местом возникновения являются почечная вена [12], почечная капсула [13] и почечная лоханка [14]. Пятнадцать процентов всех сарком мягких тканей локализуются в забрюшинном пространстве, 20 % из них имеют строение лейомиосаркомы.…”