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  and Darja Erzen 1
  • 1 Department of Surgical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
  • 2 Department of Radiology, University of Michigan Hospitals, Michigan, Ann Arbor, USA
  • 3 Department of Pathology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
  • 4 Department of Radiology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
Marko Novak
  • Corresponding author
  • Department of Surgical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
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, Andraz Perhavec
  • Department of Surgical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
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, Katherine E. Maturen
  • Department of Radiology, University of Michigan Hospitals, Ann Arbor, Michigan, USA
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, Snezana Pavlovic Djokic, Simona Jereb and Darja Erzen
  • Department of Surgical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia
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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.

Introduction

Leiomyosarcoma (LMS) is a rare malignant mesenchymal tumour of smooth muscle origin. It represents only 5–7% of soft tissue sarcomas.1Approximately 2.0% of LMS originate from the smooth muscle of vessel walls, predominantly veins and 60.0% of these originate from inferior vena cava (IVC).1 According to Gage et al. 1, the most common location of extracaval venous LMS is the renal vein, followed by the great saphenous, pulmonary and femoral vein. Leiomyosarcoma of the renal vein (LRV) is extremely rare. There have been some cases reported in the literature, but no analysis of data and search for prognostic factors have been done so far. The first case was reported by Lopez Varela and Pereira Garro in 1967.2 We present an additional case, world literature overview and the outcome of these patients.

Patients and methods

Literature overview and data collection

The search criteria in PubMed were “leiomyosarcoma” and “renal vein”. In the literature 62 articles were identified describing cases of LRV. Fourteen of the articles were in Japanese, 7 in French, 4 in Spanish, 1 in Polish and 36 in English. Data from 49 articles only2-50 were merged into a database, because out of 18 Japanese cases only 4 were reported in English articles 23,37,40,44 and the rest in Japanese articles, not accessible to us (Figure 1). The last review of Japanese cases by Kato et al.42 was translated and these data included in the study. Three times the patient was discussed as different case report by two different authors.3-4,13-14,20-26 That lowered the total number of reported cases in the last review by three.50 In some articles more than one case was reported.14,19,26,42,46 The authors were from the fields of urology (18/49; 36.7%), surgery (15/49; 30.6%), radiology (8/49; 16.3%), pathology (5/49; 10.2%) and internal medicine (3/49; 6.1%). A retrospective review was performed to evaluate patient demographics, tumour site, clinical presentation, operative details, tumour thrombus IVC extension, neoadjuvant and adjuvant treatment, tumour size, tumour grade, surgical margin status, time to local recurrence, time to dissemination, time to death and status at last follow up. To the authors or coauthors 18 emails were sent around the world to update the data and follow up, we received 4 replies.

Flow chart of identification, screening, eligibility and inclusion of studies.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Illustrative case

A 46-years old female presented in January 2014 to University Hospital Ljubljana with upper abdominal pain of 6 months duration and weight loss. Her past medical history was unremarkable. On physical examination there was a palpable mass in the left upper abdomen. Gastroscopy was not diagnostic, but computed tomography (CT) revealed a left retroperitoneal mass, 11 x 10 x 9 cm in size, interposed between the aorta and hilum of the left kidney (Figure 2). The tumour surrounded the left renal artery and the vein was not identified. Ultrasound guided fine needle aspiration biopsy (FNAB) was performed. The sample was suspicious for LMS. She was referred to the Institute of Oncology Ljubljana in February 2014 for management and treatment. A dynamic renal scintigraphy was performed for evaluation of kidney function. Excretory function of the left kidney was 47% and of the right kidney 53%. Thoracic CT revealed no metastases. After discussing the case at the multi-disciplinary team (MDT) we decided to perform surgery, without preoperative core needle biopsy (CNB). Tumour was removed en bloc with left colon, left kidney, adrenal gland and psoas fascia. A suspicious node 3 cm in size was found intraoperative in psoas muscle close to the vertebra. It was removed separately. The main specimen weighed 1152 g. Histology confirmed a LMS, according to Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grading system grade 3, 12 cm in largest diameter, originating from the left renal vein, not infiltrating the surrounding organs. Surgical margins were negative. The spindle tumour cells stained positive for smooth muscle actin, desmin and focally for CD34. The separately removed node was an LMS satellite, margins were positive (Figure 3). She received adjuvant radiotherapy (RT), 59 Gy. There were no surgical or radiotherapy-related complications. In December 2014, 10 months postoperatively, liver metastases were detected on CT. After subsequent magnetic resonance imaging treatment was planned at the MDT. All 4 liver metastases, 4 to 17 mm in size were removed surgically with clear margins. She received no adjuvant treatment. In October 2015 lung metastases were detected on both sides, the largest 17 mm. She is receiving chemotherapy (ChT) with adriamycin, ifosfamide and mesna at the time of this report.

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).

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

(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.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Written informed consent for all diagnostic and therapeutic procedures was obtained from the patient.

Statistical analysis

On the basis of limited data, univariate analysis was used to evaluate the following potential prognostic factors for overall survival (OS), local recurrence free survival (LRFS) and distant metastases free survival (DMFS): age, gender, tumour site, dissemination, weight loss, palpable mass, operation type, tumour thrombus IVC luminal extension, tumour size, grade, margin status, number of mitoses and neoadjuvant or adjuvant treatment. OS and LRFS were compared using log-rank test. All comparisons were two sided. P-value of 0.05 was considered statistically significant. Survival curves were calculated and plotted using Kaplan-Meier method. Cox′s multivariate regression was used to identify independent prognostic variables of OS, LRFS and DMFS. The statistical program SPSS® version 22 was used for analysis.

Results

Patient and tumour characteristics

In total 67 cases were identified. The tumour predominantly occurred in women (76.1%; 51/67) and on the left side (68.7%; 46/67). The mean age at diagnosis was 56.6 years (range 27–93 years). Detailed patient and clinicopathologic characteristics are presented in Table 1, Figure 4 and 5. Histological biopsy before treatment was performed in 9 patients (13.4%; 9/67); 1 patient had biopsy during exploration9, 4 patients had CT guided CNB19,20,27,50, the biopsy type for 2 patients was not specified in the article33,41 and 2 patients had biopsy through femoral approach during cavography.36,43 FNAB before operation was performed in 1 patient34 and in our case (3.0%; 22/67). The mean tumour size was 8.9 cm, described in 54 cases (80.6%; 54/67). System used for sarcoma grading was defined in single article.10 Tumour grade was described in 28 cases (41.8%; 28/67), surgical margin status in 18 cases (26.9%; 18/67) and number of mitoses in 18 cases (26.9%; 18/67). Tumour cells stained positive for smooth muscle actin in 23 cases (34.3%; 23/67), for desmin in 22 cases (32.8%;22/67) and for vimentin in 6 cases (9.0%; 6/67). Intraluminal caval tumour thrombus was reported in 9 cases (13.4%; 9/67), IVC mural invasion in 3 cases (4.5%; 3/67), the renal parenchyma invasion in 8 cases (11.9%; 8/67) and the adrenal gland invasion in a single case (1.5%; 1/67). The data about IVC mural invasion were taken as stated in the articles11,15,16.

Clinical presentation of leiomyosarcoma of the renal vein cases.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Age distribution of leiomyosarcoma of the renal vein patients.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Table 1

Patients data and histologic variables, treatment modalities and disease progression

CharacteristicSubgroupValue (n = 67)%
Age at diagnosis (year)Mean56.6
Range27–93
GenderFemale5176.1
Male1623.9
SideLeft4668.7
Right2131.3
Size (cm)Mean8.9
Range3.5–25
Tumour thrombus extensionIVC913.4
Preoperative biopsyHistology913.4
Fine needle aspiration23.0
No biopsy5683.6
Tumour gradeG169.0
G2811.9
G31420.9
Unknown3958.2
Surgical marginsNegative1522.4
Positive34.5
Unknown4973.1
OperationNephrectomy6089.6
Tumorectomy46.0
Compartment resection23.0
No operation11.5
Preoperative treatmentEmbolization34.5
ChT11.5
RT + ChT23.0
Intraoperative treatmentRT11.5
Postoperative treatmentRT710.4
ChT913.4
ChT + RT11.5
Immunotherapy11.5
Disease progressionLR34.5
M2029.9
LR + M1014.9
Total3349.3
Site of disseminationLiver1725.4
Lungs1623.9
Bone811.9
Soft tissue46.0

ChT = chemotherapy; G = grade; IVC = inferior vena cava; LR = local recurrence; M = metastases; RT = radiotherapy

Surgery

All patients but one underwent surgery (98.5%; 66/67). Four patients had tumorectomy (6.0%; 4/67) and 60 had nephrectomy (89.6%; 60/67). One patient had attempt of laparoscopic tumorectomy, two had laparoscopic nephrectomy and one had robotic laparoscopic nephrectomy. Two patients (3.0%; 2/67) had compartment resection, tumour removed en bloc with (at least) adjacent segment of colon, kidney and psoas. Adrenalectomy was performed in 11 patients (16.4%; 11/67) and lymph node dissection in 6 patients (9.0%; 6/67). Tumour thrombus extended into the lumen of IVC in 9 patients (13.4%; 9/67), in 4 cases tumour was on the left side and in 5 cases on the right. In two of these patients there was also invasion of the caval wall. IVC was resected in 5 patients (7.5%; 5/67), once ligated and without reconstruction, once oversewn, once reconstructed with venous patch and once with allograft. There are no data about the type of operation on IVC for the fifth patient. Cavotomy and extraction of the tumour thrombus was performed in 3 patients (4.5%; 3/67). One patient had locally advanced tumour, with tumour extension into the right atrium and received palliative ChT only. In a patient with tumour caval wall invasion the IVC was reconstructed with venous patch after resection. Splenectomy was performed in 2 patients, in 1 jejunal resection and in 1 synchronous liver metastatectomy.

Treatment modalities

Three patients (4.5%; 3/67) had preoperative tumour embolization. One patient received preoperative ChT and two preoperative ChT and RT (3.0%; 2/67). Seven patients (10.4%; 7/67) received postoperative RT and 9 patients postoperative ChT (13.4%; 9/67). One patient received postoperative ChT and RT and 1 patient had immunotherapy. The information about RT and ChT as neoadjuvant and adjuvant treatment is summarized in Table 1.

Outcome

Four patients were excluded for the survival analysis, because 3 were disseminated at the time of diagnosis 19,20,26 and one was not treated surgically.36 Two patients were alive with disease and on palliative care at the time of report.34,46 Three patients (4.5%; 3/67) had local recurrence, 10 patients (14.9%; 10/67) had local recurrence and dissemination and 20 patients (29.9%; 20/67) had dissemination of the disease after treatment. Spread was hematogenous to different organs. In this group of 67 patients to the liver in 25.4% (17/67), lungs in 23.9% (16/67), bones in 11.9% (8/67) and soft tissue in 6.0% (4/67) (Table 1).

After the median follow up of 24 months, the OS was 79.5%. LRFS was 83.5% after median follow up of 21.5 months and DMFS was 76.1% after median follow up of 22 months. Factors predictive of OS in univariate analysis were surgical margins (p = 0.014), while factors predictive of LRFS in univariate analysis were IVC luminal extension (p = 0.016) and tumour grade (p = 0.05). No factors predictive of DMFS were identified in univariate analysis. Univariate analysis of OS, LRFS and DMFS are presented in Table 2. In multivariate analysis none of the factors were predictive of OS, LRFS or DMFS. Survival curves are presented in Figures 6, 7 and 8.

Kaplan-Meier curve of overall freesurvival.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Kaplan-Meier curve of local recurrence free survival.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Kaplan-Meier curve of distant metastases free survival.

Citation: Radiology and Oncology 51, 1; 10.1515/raon-2016-0051

Discussion

Points to be discussed about the case from our institution are biopsy, surgery, adjuvant RT and treatment of liver metastases.

The patient presented to the Institute of Oncology Ljubljana because of retroperitoneal location of the tumour and cytological suspicion for LMS. Ultrasound guided FNAB was performed in another hospital. At the MDT it was not decided for CNB, because the mass was a spindle cell tumour, suspicious for LMS, with the renal vein not identified on CT, indeed suspicious for primary LRV, and because the tumour was deemed resectable and not disseminated, as such not planned for neoadjuvant treatment.

The tumour was removed with compartment resection with negative margins, in separately removed satellite margins were positive. Analysing the CT scans again after the histological report, the satellite was found on CT and it seems that the tumour was invading the psoas muscle in continuity. Surgery was planned as wide resection but was marginal and R1. The operation would be optimal if both specimens would be removed en bloc, but the margins on the vertebra would probably be positive anyway. Because reoperation with clear margins on vertebra in case of local recurrence would probably not be possible, we decided for adjuvant RT.

Table 2

Univariate analysis of overall, local recurrence free and distant metastases free survival (log-rank)

CharacteristicSubgroupOVERALL SURVIVALLOCAL RECURRENCE FREE SURVIVAL
Alive (n = 39)Dead (n = 17)p-valueNo LR (n = 45)LR (n = 11)p-value
Age≤ 501350.2851530.519
502612308
GenderF32150.8123890.993
M7272
SideL2990.4483170.987
R108144
Weight lossY560.414920.734
N3411369
Palpable massY960.5811240.562
N3011349
OperationNephrectomy35150.5434190.562
Tumorectomy22(nephrectomy or tumorectomy vs. compartment)22
Compartment2020
Intracaval luminal extensionY530.340440.016
N3414417
Tumour size≤ 10 cm23100.4852850.219
> 10 cm113(≤ 10 cm vs. > 10 cm)113(≤ 10 cm vs. > 10 cm)
Unknown5463
Grade1420.265 (1 vs. 2 or 3)600.05 (1 vs 2 or 3)
2 or 3136127
Unknown229274
MarginsNegative1410.014 (neg. vs. pos.)1230.096 (neg. vs. pos.)
Positive1102
Unknown2415336
Mitoses/10hpf<10820.782 (<10 vs. ≥10)820.244 (<10 vs. ≥10)
≥102112
Unknown2914367
Neoadjuvant/adjuvant chemotherapyY830.987740.337
N3114387
Neoadjuvant/adjuvant raditherapyY640.165820.975
N3313379

F = female; L = left; LR = local recurrence; M = male; N = No; R = right; Y = Yes

CharacteristicSubgroupDISTAN1 METASTASES FREE SURVIVAL
No DM (n = 31)DM (n = 25)p-value
Age≤ 50990.805
> 502216
GenderF27200.221
M45
SideL19190.138
R126
Weight lossY290.087
N2916
Palpable massY5100.277
N2615
OperationNephrectomy27230.336
Tumorectomy31(nephrectomy or tumorectomy vs. compartment)
Compartment11
Intracaval luminal extensionY440.284
N2721
Tumour size≤ 10 cm21120.210
> 10 cm77(≤ 10 cm vs. > 10 cm)
Unknown36
Grade1420.131 (1 vs. 2 or 3)
2 or 3910
Unknown1813
MarginsNegative1050.815 (neg. vs. pos.)
Positive11
Unknown2019
Mitoses/10hpf<10910.266 (<10 vs. ≥10)
≥1012
Unknown2122
Neoadjuvant/adjuvant chemotherapyY560.683
N2619
Neoadjuvant/adjuvant radiotherapyY550.087
N2620

DM = distant metastases; F = female; L = left; M = male; N = No; R = right; Y = Yes

According to magnetic resonance imaging liver metastases were small and resectable and that was the reason at the MDT to decide for metastasectomy.

LRV is very rare. Cases from the last literature overview in 2010 50, cases from nonenglish literature, new reports from 2010–2015 and present case were summarised. From data gathered from these case reports, subsequent analysis and with respect to sarcoma guidelines, several observations can be made.

From the clinical point of view, LRV presents difficulties in making diagnosis, because it is uncommon, has no specific symptoms and no pathognomonic radiological features. It predominantly occurs in women (76.1%), on the left side (68.7%) and affects older population, with the peak occurring at age 60–69 years. Presenting symptoms are unspecific, abdominal pain was reported in 49.3%. Hematuria was reported in a single case (1.5%) of LRV patients, but is present in more than one third of the cases (34.8%) of renal cell carcinoma (RCC) with venous extension.51 Genetic predisposition may play a role in development of primary LRV, with two patients being treated for retinoblastoma and one patient having Li Fraumeni syndrome.

From the point of imaging, location of LRV is more important than the size of the tumour. It can overlap with much more common RCC with venous extension. LRV is usually located in the hilum of the kidney. The bulk of the tumour lies predominantly or entirely outside the hilar parenchyma or the tumour is limited to the renal vessels [46]. The mean tumour size in this LRV group is 8.9 cm. In a study group of 1192 patients with RCC with extension into the renal vein (23.0%) and IVC (7.0%) the mean tumour size was 8.9 cm as well.52 It may not be possible to distinguish between these two entities by imaging. Other diagnoses considered in this location are metastatic lymph node in a patient with a history of malignancy, renal pelvis leiomyosarcoma, extremely rare as well, with around 10 cases reported in the literature53, lymphoma, adrenal gland tumour, upper tract urothelial carcinoma, granulomatous disease and renal vein thrombus.46

With regard to biopsy, retroperitoneal mass is usually detected on abdominal CT scans. When imaging is not diagnostic of a retroperitoneal liposarcoma, image-guided CNB of retroperitoneal tumour is strongly recommended to obtain the sample for diagnosis. Correct diagnosis may significantly affect surgical decision and neo/adjuvant therapy.54,55 Wilkinson et al.56 from Royal Marsden, London reported, that preoperative CNB for retroperitoneal sarcoma (RPS) is safe and does not affect oncological outcome. Patients with intermediate and high-grade RPS were included. There were no intra-abdominal complications requiring early operation. The group of 90 patients with preoperative CNB was compared to a group of 60 patients, who did not have preoperative CNB. There was no significant difference in local recurrence (p = 0.101) or OS (p = 0.191). FNAB in retroperitoneal tumours rarely yields diagnostic information and should be avoided55, but it can be performed in RCC in spite of danger of haemorrhage. In the present review preoperative histological biopsy was performed in 13.4% of cases only and FNAB in 3.0%.

With regard to treatment, the only potentially curative treatment for RPS is surgery with macroscopically complete resection.54,55,57 The role of ChT and RT in RPS is not proven and still under investigation. It is generally recommended, that in case of RT administration, it should be delivered in the preoperative setting and possibly within a clinical trial.54 Postoperative RT should not be administered routinely in R0 and R1 resections.54 ChT is an option in the preoperative setting of resectable disease, is an option after surgery in case of R2 resection and is an option in case of unresectable or metastatic disease.54

Because of complex evaluation and treatment options patients with RPS should be managed by sarcoma MDT in a specialized reference center.54,55

Histologic subtype is one of the major determinants of the oncologic outcome in RPS. The most common location of LMS is the retroperitoneum58, where it represents the second most common histological subtype after liposarcoma, accounting for 14–36% of patients in major series.59,60 Retroperitoneal LMS has a high propensity for distant recurrence. The reported rate of distant metastases for retroperitoneal LMS at 5 years is around 40–50% and for local recurrence at 5 years around 5%.61 Similar results are present in the present review, with the rate of local recurrence of 4.5% (3/67), distal metastases of 29.9% (20/67) and both in 14.9% (10/67), but in much shorter period of follow up.

And finally, in the present review of the literature 79.5% of the LRV patients survived at 2 years. A 5-year OS, LRFS and DMFS was not performed because of the inadequate sample size at that length of follow up. Retrospective comparisons of series of RPS patients have demonstrated 5-years OS rates of 50–70% and 5-years local control rates of 40–80%.57 In the IVC LMS series 5-year survival has been reported between 33.0% and 53.0%.26 Data from different large series of RPS patients have demonstrated tumour grade and surgical margin status as independent prognostic factors of OS and LRFS.62,63 Cases from this review are dispersed world wide and through half of the century, lacking data for tumour grade (58.2%; 39/67), surgical margin status (73.1%; 49/67) and follow up (16.4%; 11/67). Because of insufficient histologic data and truncated follow up, we were not able to identify prognostic factors of OS, LRFS and DMFS in multivariate analysis.

As a retrospective analysis this study has limitations. Most of the information collected was from case reports, without significant follow up and lacking histological data. As a consequence, there was a limitation in the statistical analysis and the conclusions that could be drawn from it, particularly in patients’ outcome. However, to our knowledge, this is the largest study on this topic, and even this limited survey expands our understanding of the natural history of this rare sarcoma.

Conclusions

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 of 40, on the left side and in the absence of hematuria. Core needle biopsy should be performed. Patients should be managed by sarcoma MDT. For optimal clinical outcomes, LRV should be surgically removed, with negative margins. After a median follow up of 24 months 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. Because of insufficient histologic data and follow up, we were not able to identify prognostic factors of OS, LRFS and DMFS in multivariate analysis.

Acknowledgements

The authors gratefully acknowledge Dr. Gideon Adam Blecher, Dr. Mark Frydenberg, Dr. Yosuke Ikegami, Dr. Wojciech Wysocky, Dr. Zbigniew Darasz and Dr. Raghu Vikram for assistance with patients data collection. Sincere thanks to Matjaž Musek, Ksenija Žmavc and the Library of the Institute of Oncology Ljubljana for providing the articles, to Jure Čižman for preparing the figures and to Ikue Nishi for translation from Japanese.

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  • 23

    Inoue K, Watanabe H, Ohashi Y, Morioka M, Fujita Y. Leiomyosarcoma of the renal vein: a case report. J Urol 1994; 152: 153-5.

    • Crossref
    • PubMed
    • Export Citation
  • 24

    Lipton M, Sprayregen S, Kutcher R, Frost A. Venous invasion in renal vein leiomyosarcoma: case report and review of the literature. Abdom Imaging 1995; 20: 64-7.

    • Crossref
    • PubMed
    • Export Citation
  • 25

    Alcover Garcia J, Ramirez Ruz J, Umbert Canals B, Carrere Springli W, Fernandez-Conde Montoya M, Carretero Gonzalez P. Leiomyosarcoma originating in the renal vein. A special case. Arch Esp Urol 1996; 49: 638-42.

    • PubMed
    • Export Citation
  • 26

    Brandes SB, Chelsky MJ, Petersen RO, Greenberg RE. Leiomyosarcoma of the renal vein. J Surg Oncol 1996; 63: 195-200.

    • Crossref
    • PubMed
    • Export Citation
  • 27

    Polsky S, Goodloe S Jr, Peterson S, Karakousis CP. Leiomyosarcoma of the renal vein. Eur J Surg Oncol 1997; 23: 456.

    • Crossref
    • PubMed
    • Export Citation
  • 28

    Maglione M, Maglione O, Sonzini Astudillo C, Travecchio J, Giraudo R. Leiomyosarcoma of the renal vein. A case of exception. Rev Arg de Urol 1999; 64: 199-201.

  • 29

    Soulie M, Connan L, Escourrou G, Seguin P, Pontonnier F, Plante P. Leiomyosarcoma of the renal vein. Prog Urol 2001; 11: 502-6.

    • PubMed
    • Export Citation
  • 30

    Hiratuka Y, Ikeda H, Sugaya Y, Tozuka K, Yamada S. A case of leiomyosarcoma of the renal vein. Nihon Hinyokika Gakkai Zasshi 2001; 92: 38-41.

  • 31

    Kaushik S, Neifeld JP. Leiomyosarcoma of the renal vein: imaging and surgical reconstruction. AJR Am J Roentgenol 2002; 179: 276-7.

    • Crossref
    • PubMed
    • Export Citation
  • 32

    Lemos GC, El Hayek OR, Apezzato M. Leiomyosarcoma of the renal vein. Int Braz J Urol 2003; 29: 43-4.

    • Crossref
    • PubMed
    • Export Citation
  • 33

    Kolodziejski LS, Dyczek ST, Gruchala A, Darasz Z, Marczyk E. Resection of the renal vein leiomyosarcoma with preservation of the kidney. Przegl Lek 2004; 61: 2002-4.

  • 34

    Colon-Rodriguez A, Bernardos-Garcia L, Calleja-Kempin J, Reparaz L, Gimeno-Aranguez M, Martinez-Baena D. Leiomiosarcoma de la vena renal izquierda. Angiologia 2004; 56: 75-80.

    • Crossref
    • Export Citation
  • 35

    Aguilar IC, Benavente VA, Pow-Sang MR, Morante CM, Meza L, Destefano V, et al. Leiomyosarcoma of the renal vein: case report and review of the literature. Urol Oncol 2005; 23: 22-6.

    • Crossref
    • PubMed
    • Export Citation
  • 36

    Mansencal N, El Hajjam M, Vieillard-Baron A, Pelage JP, Lacombe P, Dubourg O. Recurrent pulmonary embolism with non-mobile thrombus in a patient with leiomyosarcoma of the left renal vein. Int J Cardiol 2006: 112: 247-8.

    • Crossref
    • Export Citation
  • 37

    Maeda T, Tateishi U, Fujimoto H, Kanai Y, Sugimura K, Arai Y. Leiomyosarcoma of the renal vein: arterial encasement on contrast-enhanced dynamic computed tomography. Int J Urol 2006; 13: 611-2.

    • Crossref
    • PubMed
    • Export Citation
  • 38

    Mssrouri R, Khalid Lahlou M, Benamr S, Essadel A, Mdaghri J, Mohammadine EH, et al. Leiomyosarcome primitif de la veine renale. Feuillets de Radiologie 2006; 46: 457-61.

    • Crossref
    • Export Citation
  • 39

    Nese N, Cavdar KD, Gumus B, Isisag A. Low-grade leiomyosarcoma of renal vein: A case report. Turk Patoloji Dergisi 2008; 24: 50-3.

  • 40

    Ikegami Y, Umemoto Y, Kohri K. Leiomyosarcoma of the renal vein. Int J Urol 2009; 16: 768.

    • Crossref
    • PubMed
    • Export Citation
  • 41

    Gonzalez-Rodriguez FJ, Punal-Rodriguez JA, Paredes-Cotore JP, Beiras A. Leiomiosarcoma de la vena renal. Discusion de su manejo terapeutico. Cir Esp 2009; 86: 111-21.

    • Crossref
    • Export Citation
  • 42

    Kato T, Yoneda S, Madono K, Tanigawa G, Fujita K, Yazawa K, et al. Leiomyosarcoma of the renal vein: a case report. Hinyokika Kiyo 2009; 55: 607-10.

    • PubMed
    • Export Citation
  • 43

    Houitte R, Jousset Y, Delori M, Abgueguen P, Tanguy M, Fanello S. Renal leiomyosarcoma, rheumatoid arthritis and methotrexate. J Mal Vasc 2010; 35: 194-6.

    • PubMed
    • Export Citation
  • 44

    Imao T, Amano T, Takemae K. Leiomyosarcoma of the renal vein. Int J Clin Oncol 2011; 16: 76-9.

    • Crossref
    • PubMed
    • Export Citation
  • 45

    Douma S, Kamparoudis A, Petidis K, Anyfanti P, Doumas M, Gkaliagkousi E, et al. Leiomyosarcoma of renal vein, initially resembling pheochromocytoma. Clin Exp Hypertens 2012; 34: 429-31.

    • Crossref
    • PubMed
    • Export Citation
  • 46

    Maturen KE, Vikram R, Wu AJ, Francis IR. Renal vein leiomyosarcoma: imaging and clinical features of a renal cell carcinoma mimic. Abd Imag 2013; 38: 379-87.

    • Crossref
    • Export Citation
  • 47

    Saltzman FA, Brown TE, Halat KS, Hedgepeth CR. An uncommonly encountered perirenal mass: robotic resection of renal vein leiomyosarcoma. Can Urol Assoc J 2015; 9: E213-6.

    • Crossref
    • PubMed
    • Export Citation
  • 48

    Chougule A, Bal A, Kumar Mandal A. Primary renal vein leiomyosarcoma: a case report, Cardiovasc Pathol (2015), http://dx.doi.org/10.1016/j.carpath.2015.05.002.

    • PubMed
    • Export Citation
  • 49

    Devlin M, Gill K, Thomas J, Biyani CS. Renal vein leiomyosarcoma and renal cell carcinoma presenting together: A case report and discussion on the follow-up. Can Urol Assoc J 2015; 9: E517-20.

    • Crossref
    • PubMed
    • Export Citation
  • 50

    Frydenberg M, Blecher GA. Case reports: renal vein leiomyosarcoma. BJU Int (serial online). Available from: http://www.bjui.org/Content-Fullltem.aspx?id=559&SectionType=1. Accessed 15 March 2015.

  • 51

    Lambert HE, Pierorazio MP, Shabsigh A, Olsson AC, Benson CM, McKiernan MJ. Prognostic risk stratification and clinical outcomes in patients undergoing surgical treatment for renal cell carcinoma with vascular tumor thrombus. J Urol 2007; 69: 1054-9.

    • Crossref
    • Export Citation
  • 52

    Wagner B, Patard JJ, Mejean A, Bensalah K, Verhoest G, Zigeuner R, et al. Prognostic value of renal vein and inferior vena cava involvement in renal cell carcinoma. Eur Urol 2009; 55: 452-60.

    • Crossref
    • PubMed
    • Export Citation
  • 53

    Dhamne SA, Gadgil NM, Padmanabhan A. Leiomyosarcoma of the renal pelvis. Indian J Pathol Microbiol 2009; 52: 549-51.

    • Crossref
    • PubMed
    • Export Citation
  • 54

    National Comprehensive Cancer Network (NCCN) Practice guidelines in oncology: Soft tissue sarcoma – retroperitoneal/intraabdominal. Available from http://www.globalgist.org/docs/NCCN_guidelines.pdf. Accessed 15 April 2015.

  • 55

    Trans-Atlantic RPS Working Group. Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS working group. Ann Surg Oncol 2015; 22: 256-63.

    • PubMed
    • Export Citation
  • 56

    Wilkinson MJ, Martin JL, Khan AA, Hayes AJ, Thomas JM, Strauss DC. Percutaneous core needle biopsy in retroperitoneal sarcomas does not influence local recurrence or overall survival. Ann Surg Oncol 2015; 22: 853-8.

    • Crossref
    • PubMed
    • Export Citation
  • 57

    Kneisl JS, Coleman MM, Raut CP. Outcomes in the management of adult soft tissue sarcomas. J Surg Oncol 2014; 110: 527-38.

    • Crossref
    • PubMed
    • Export Citation
  • 58

    Fletcher CDM, Bridge JA, Hogendoorm PCW, Mertens F. WHO classification of tumours of soft tissue and bone. 4th edition. Lyon: International Agency for Research on Cancer (IARC); 2013.

  • 59

    Gronhi A, Lo Vullo S, Fiore M, Mussi C, Stacchiotti S, Collini P, et al. Aggressive surgical policies in a retrospectively reviewed single-institution case series of retroperitoneal soft tissue sarcoma patients. J Clin Oncol 2009; 27: 24-30.

    • Crossref
    • Export Citation
  • 60

    Erzen D, Sencar M, Novak J. Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana. J Surg Oncol 2005; 91: 1-9.

    • Crossref
    • PubMed
    • Export Citation
  • 61

    Crago AM. Extended surgical resection and histology in retroperitoneal sarcoma. Ann Surg Oncol 2015; 22: 1401-3.

    • Crossref
    • PubMed
    • Export Citation
  • 62

    Bonvalot S, Rivoire M, Castaing M, Stoeckle E, Le Cesne A, Blay JY, et al. Primary retroperitoneal sarcomas: a multivariate analysis of surgical factors associated with local control. J Clin Oncol 2009; 27: 31-7.

    • Crossref
    • PubMed
    • Export Citation
  • 63

    Bremjit PJ, Jones RL, Chai X, Kane G, Rodler ET, Loggers ET, et al. A contemporary large single-institution evaluation of resected retroperitoneal sarcoma. Ann Surg Oncol 2014; 21: 2150-8.

    • Crossref
    • PubMed
    • Export Citation

If the inline PDF is not rendering correctly, you can download the PDF file here.

  • 1

    Gage MJ, Patel AV, Koenig KL, Newman E. Non-vena cava venous leiomyosarcoma: a review of the literature. Ann Surg Oncol 2012; 19: 3368-74.

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    Kaufman JJ, Gelbard M. Leiomyosarcoma of renal vein and inferior vena cava. Urology 1981; 18: 173-6.

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    Dufour B, Choquenet C, Nacash G. Primary leiomyosarcoma of the right renal vein with extension into the inferior vena cava. J Urol (Paris) 1982; 88: 561-5.

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  • 12

    Hisa A, Hirakawa K, Kamioka N. A case of primary renal vein leiomyosarcoma. Med J Kochi Municipal Centre Hosp 1984; 11: 53-7.

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    Farges O, Gugenheim J, Pascal G, Pujol JP, Roche A, Bismuth H. Primary leiomyosarcoma of the renal vein. Ann Chir 1986; 40: 493-7.

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  • 14

    Martin B, Roche A, Menu Y. Radiologic diagnosis of leiomyosarcomas of the renal vein: the role of angiography. A review of the literature apropos of 2 new cases. J Radiol 1986; 67: 789-95.

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    Phoa SS, van Rooij WJ, Kox C, Dijkstra PF. Leiomyosarcoma of the suprarenal and renal veins, report on two cases. Rofo 1988; 148: 84-5.

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    Vos P, Barwegen MG, Bakker HH, Dabhoiwala NF, Schipper ME. Leiomyosarcoma of the renal vein: a case report. J Urol 1988; 139: 1042-4.

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  • 17

    Martin J, Garcia M, Duran A, Forcada P, Marco V. Renal vein leiomyosarcoma: a case report and literature review. Urol Radiol 1989; 11: 25-9.

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    • PubMed
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  • 18

    Farah MC, Shirkhoda A, Ellwood RA, Bernacki E, Farah J. Leiomyosarcoma of the renal vein: radiologic pathologic correlation. Clin Imaging 1989; 13: 323-6.

    • Crossref
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  • 19

    Ball AB, Fisher C. Leiomyosarcoma of the renal vein: a report of two cases. Eur Urol 1990; 18: 150-2.

    • Crossref
    • PubMed
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  • 20

    Pelton JJ, Palazzo JP, Peterson RO, Eisenberg BL. Renal vein leiomyosarcoma. J Surg Oncol 1990; 45: 131-3.

    • Crossref
    • PubMed
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  • 21

    Grignon DJ, Ro JY, Papadopoulos NE, Ayala AG. Leiomyosarcoma of renal vein. Urology 1991; 38: 255-8.

    • Crossref
    • PubMed
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  • 22

    Lakhloufi A, Khaiz D, Abi F, Bouzidi A. Primary leiomyosarcoma of the left renal vein. Ann Urol (Paris) 1992; 26: 333-5.

    • PubMed
    • Export Citation
  • 23

    Inoue K, Watanabe H, Ohashi Y, Morioka M, Fujita Y. Leiomyosarcoma of the renal vein: a case report. J Urol 1994; 152: 153-5.

    • Crossref
    • PubMed
    • Export Citation
  • 24

    Lipton M, Sprayregen S, Kutcher R, Frost A. Venous invasion in renal vein leiomyosarcoma: case report and review of the literature. Abdom Imaging 1995; 20: 64-7.

    • Crossref
    • PubMed
    • Export Citation
  • 25

    Alcover Garcia J, Ramirez Ruz J, Umbert Canals B, Carrere Springli W, Fernandez-Conde Montoya M, Carretero Gonzalez P. Leiomyosarcoma originating in the renal vein. A special case. Arch Esp Urol 1996; 49: 638-42.

    • PubMed
    • Export Citation
  • 26

    Brandes SB, Chelsky MJ, Petersen RO, Greenberg RE. Leiomyosarcoma of the renal vein. J Surg Oncol 1996; 63: 195-200.

    • Crossref
    • PubMed
    • Export Citation
  • 27

    Polsky S, Goodloe S Jr, Peterson S, Karakousis CP. Leiomyosarcoma of the renal vein. Eur J Surg Oncol 1997; 23: 456.

    • Crossref
    • PubMed
    • Export Citation
  • 28

    Maglione M, Maglione O, Sonzini Astudillo C, Travecchio J, Giraudo R. Leiomyosarcoma of the renal vein. A case of exception. Rev Arg de Urol 1999; 64: 199-201.

  • 29

    Soulie M, Connan L, Escourrou G, Seguin P, Pontonnier F, Plante P. Leiomyosarcoma of the renal vein. Prog Urol 2001; 11: 502-6.

    • PubMed
    • Export Citation
  • 30

    Hiratuka Y, Ikeda H, Sugaya Y, Tozuka K, Yamada S. A case of leiomyosarcoma of the renal vein. Nihon Hinyokika Gakkai Zasshi 2001; 92: 38-41.

  • 31

    Kaushik S, Neifeld JP. Leiomyosarcoma of the renal vein: imaging and surgical reconstruction. AJR Am J Roentgenol 2002; 179: 276-7.

    • Crossref
    • PubMed
    • Export Citation
  • 32

    Lemos GC, El Hayek OR, Apezzato M. Leiomyosarcoma of the renal vein. Int Braz J Urol 2003; 29: 43-4.

    • Crossref
    • PubMed
    • Export Citation
  • 33

    Kolodziejski LS, Dyczek ST, Gruchala A, Darasz Z, Marczyk E. Resection of the renal vein leiomyosarcoma with preservation of the kidney. Przegl Lek 2004; 61: 2002-4.

  • 34

    Colon-Rodriguez A, Bernardos-Garcia L, Calleja-Kempin J, Reparaz L, Gimeno-Aranguez M, Martinez-Baena D. Leiomiosarcoma de la vena renal izquierda. Angiologia 2004; 56: 75-80.

    • Crossref
    • Export Citation
  • 35

    Aguilar IC, Benavente VA, Pow-Sang MR, Morante CM, Meza L, Destefano V, et al. Leiomyosarcoma of the renal vein: case report and review of the literature. Urol Oncol 2005; 23: 22-6.

    • Crossref
    • PubMed
    • Export Citation
  • 36

    Mansencal N, El Hajjam M, Vieillard-Baron A, Pelage JP, Lacombe P, Dubourg O. Recurrent pulmonary embolism with non-mobile thrombus in a patient with leiomyosarcoma of the left renal vein. Int J Cardiol 2006: 112: 247-8.

    • Crossref
    • Export Citation
  • 37

    Maeda T, Tateishi U, Fujimoto H, Kanai Y, Sugimura K, Arai Y. Leiomyosarcoma of the renal vein: arterial encasement on contrast-enhanced dynamic computed tomography. Int J Urol 2006; 13: 611-2.

    • Crossref
    • PubMed
    • Export Citation
  • 38

    Mssrouri R, Khalid Lahlou M, Benamr S, Essadel A, Mdaghri J, Mohammadine EH, et al. Leiomyosarcome primitif de la veine renale. Feuillets de Radiologie 2006; 46: 457-61.

    • Crossref
    • Export Citation
  • 39

    Nese N, Cavdar KD, Gumus B, Isisag A. Low-grade leiomyosarcoma of renal vein: A case report. Turk Patoloji Dergisi 2008; 24: 50-3.

  • 40

    Ikegami Y, Umemoto Y, Kohri K. Leiomyosarcoma of the renal vein. Int J Urol 2009; 16: 768.

    • Crossref
    • PubMed
    • Export Citation
  • 41

    Gonzalez-Rodriguez FJ, Punal-Rodriguez JA, Paredes-Cotore JP, Beiras A. Leiomiosarcoma de la vena renal. Discusion de su manejo terapeutico. Cir Esp 2009; 86: 111-21.

    • Crossref
    • Export Citation
  • 42

    Kato T, Yoneda S, Madono K, Tanigawa G, Fujita K, Yazawa K, et al. Leiomyosarcoma of the renal vein: a case report. Hinyokika Kiyo 2009; 55: 607-10.

    • PubMed
    • Export Citation
  • 43

    Houitte R, Jousset Y, Delori M, Abgueguen P, Tanguy M, Fanello S. Renal leiomyosarcoma, rheumatoid arthritis and methotrexate. J Mal Vasc 2010; 35: 194-6.

    • PubMed
    • Export Citation
  • 44

    Imao T, Amano T, Takemae K. Leiomyosarcoma of the renal vein. Int J Clin Oncol 2011; 16: 76-9.

    • Crossref
    • PubMed
    • Export Citation
  • 45

    Douma S, Kamparoudis A, Petidis K, Anyfanti P, Doumas M, Gkaliagkousi E, et al. Leiomyosarcoma of renal vein, initially resembling pheochromocytoma. Clin Exp Hypertens 2012; 34: 429-31.

    • Crossref
    • PubMed
    • Export Citation
  • 46

    Maturen KE, Vikram R, Wu AJ, Francis IR. Renal vein leiomyosarcoma: imaging and clinical features of a renal cell carcinoma mimic. Abd Imag 2013; 38: 379-87.

    • Crossref
    • Export Citation
  • 47

    Saltzman FA, Brown TE, Halat KS, Hedgepeth CR. An uncommonly encountered perirenal mass: robotic resection of renal vein leiomyosarcoma. Can Urol Assoc J 2015; 9: E213-6.

    • Crossref
    • PubMed
    • Export Citation
  • 48

    Chougule A, Bal A, Kumar Mandal A. Primary renal vein leiomyosarcoma: a case report, Cardiovasc Pathol (2015), http://dx.doi.org/10.1016/j.carpath.2015.05.002.

    • PubMed
    • Export Citation
  • 49

    Devlin M, Gill K, Thomas J, Biyani CS. Renal vein leiomyosarcoma and renal cell carcinoma presenting together: A case report and discussion on the follow-up. Can Urol Assoc J 2015; 9: E517-20.

    • Crossref
    • PubMed
    • Export Citation
  • 50

    Frydenberg M, Blecher GA. Case reports: renal vein leiomyosarcoma. BJU Int (serial online). Available from: http://www.bjui.org/Content-Fullltem.aspx?id=559&SectionType=1. Accessed 15 March 2015.

  • 51

    Lambert HE, Pierorazio MP, Shabsigh A, Olsson AC, Benson CM, McKiernan MJ. Prognostic risk stratification and clinical outcomes in patients undergoing surgical treatment for renal cell carcinoma with vascular tumor thrombus. J Urol 2007; 69: 1054-9.

    • Crossref
    • Export Citation
  • 52

    Wagner B, Patard JJ, Mejean A, Bensalah K, Verhoest G, Zigeuner R, et al. Prognostic value of renal vein and inferior vena cava involvement in renal cell carcinoma. Eur Urol 2009; 55: 452-60.

    • Crossref
    • PubMed
    • Export Citation
  • 53

    Dhamne SA, Gadgil NM, Padmanabhan A. Leiomyosarcoma of the renal pelvis. Indian J Pathol Microbiol 2009; 52: 549-51.

    • Crossref
    • PubMed
    • Export Citation
  • 54

    National Comprehensive Cancer Network (NCCN) Practice guidelines in oncology: Soft tissue sarcoma – retroperitoneal/intraabdominal. Available from http://www.globalgist.org/docs/NCCN_guidelines.pdf. Accessed 15 April 2015.

  • 55

    Trans-Atlantic RPS Working Group. Management of primary retroperitoneal sarcoma (RPS) in the adult: a consensus approach from the Trans-Atlantic RPS working group. Ann Surg Oncol 2015; 22: 256-63.

    • PubMed
    • Export Citation
  • 56

    Wilkinson MJ, Martin JL, Khan AA, Hayes AJ, Thomas JM, Strauss DC. Percutaneous core needle biopsy in retroperitoneal sarcomas does not influence local recurrence or overall survival. Ann Surg Oncol 2015; 22: 853-8.

    • Crossref
    • PubMed
    • Export Citation
  • 57

    Kneisl JS, Coleman MM, Raut CP. Outcomes in the management of adult soft tissue sarcomas. J Surg Oncol 2014; 110: 527-38.

    • Crossref
    • PubMed
    • Export Citation
  • 58

    Fletcher CDM, Bridge JA, Hogendoorm PCW, Mertens F. WHO classification of tumours of soft tissue and bone. 4th edition. Lyon: International Agency for Research on Cancer (IARC); 2013.

  • 59

    Gronhi A, Lo Vullo S, Fiore M, Mussi C, Stacchiotti S, Collini P, et al. Aggressive surgical policies in a retrospectively reviewed single-institution case series of retroperitoneal soft tissue sarcoma patients. J Clin Oncol 2009; 27: 24-30.

    • Crossref
    • Export Citation
  • 60

    Erzen D, Sencar M, Novak J. Retroperitoneal sarcoma: 25 years of experience with aggressive surgical treatment at the Institute of Oncology, Ljubljana. J Surg Oncol 2005; 91: 1-9.

    • Crossref
    • PubMed
    • Export Citation
  • 61

    Crago AM. Extended surgical resection and histology in retroperitoneal sarcoma. Ann Surg Oncol 2015; 22: 1401-3.

    • Crossref
    • PubMed
    • Export Citation
  • 62

    Bonvalot S, Rivoire M, Castaing M, Stoeckle E, Le Cesne A, Blay JY, et al. Primary retroperitoneal sarcomas: a multivariate analysis of surgical factors associated with local control. J Clin Oncol 2009; 27: 31-7.

    • Crossref
    • PubMed
    • Export Citation
  • 63

    Bremjit PJ, Jones RL, Chai X, Kane G, Rodler ET, Loggers ET, et al. A contemporary large single-institution evaluation of resected retroperitoneal sarcoma. Ann Surg Oncol 2014; 21: 2150-8.

    • Crossref
    • PubMed
    • Export Citation
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  • View in gallery

    Flow chart of identification, screening, eligibility and inclusion of studies.

  • View in gallery

    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).

  • View in gallery

    (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.

  • View in gallery

    Clinical presentation of leiomyosarcoma of the renal vein cases.

  • View in gallery

    Age distribution of leiomyosarcoma of the renal vein patients.

  • View in gallery

    Kaplan-Meier curve of overall freesurvival.

  • View in gallery

    Kaplan-Meier curve of local recurrence free survival.

  • View in gallery

    Kaplan-Meier curve of distant metastases free survival.