scholarly journals Leiomyosarcoma of the Inferior Vena Cava: A Case Report of Complete and Sustained Response with Trabectedin

2016 ◽  
Vol 29 (4) ◽  
pp. 284 ◽  
Author(s):  
André Cruz ◽  
Luís Bretes ◽  
Carlos Reis ◽  
Irene Furtado

<p>Inferior vena cava leiomyosarcoma is a very rare tumor, accounting for only 0.5% of all soft tissue sarcomas. As the other leyomiosarcomas of vascular origin, they have a poor prognosis, and radical resection with surgical margins free of tumor is the only potentially curative treatment. We present a case of a 46 year-old woman with metastatic inferior vena cava leiomyosarcoma who progressed after anthracyclines and ifosfamide and achieved a complete and sustained response with trabectedin. Beyond progression, the patient started third line treatment with pazopanib. A brief review of literature is also given. This case supports the efectiveness of a recent therapeutic agent, with an impressive progression-free survival in a recurrent metastatic inferior vena cava leiomyosarcoma.</p>

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10572-10572
Author(s):  
Alessandro Comandone ◽  
Antonella Boglione ◽  
Elena Giubellino ◽  
Paola Bergnolo ◽  
Giancarlo Gino ◽  
...  

10572 Background: In STS third line treatment is poorly defined. However many patients (pts), after aggressive therapy as first and second line progress in their disease ask to be treated. Oral cyclophosphamide (CPM) was already used in breast cancer, prostate cancer and in elderly pts with STS with favourable results. Aim of our study was to define the feasibility, tolerability and activity of oral CPM as third line and further line chemotherapy Methods: 45 pts (19 M; 26 F) with advanced or metastatic STS heavily pretreated were included. Oral CPM was given daily at total dose of 50 mg/day without interruption excepted for toxicity or progressive disease Results: Median age was 60 (32-81), histological subtypes were: leiomyosarcoma 12, liposarcoma 10, condrosarcoma 5, sinovialsarcoma 4, sarcoma NOS 4, other 10. Primary sites were: extremities 21, retroperitoneum 19, trunk 5. 41 pts were metastatic, 4 locally advanced. 41 pts were pretreated with chemotheraphy (15 were in II line, 17 in III line, 7 in IV line, 2 in V line). Median PS (ECOG) was 2 . Median duration of theraphy was 4 months (1-38). Progression free survival (PFS) ranged from 0 to 42+ months (median 4 months). Treatment was well tolerated, we registred only one episode of leucopenia G2 and one of asthenia G2. No complete responses were seen. Only 3 minimal responses and 18 stable disease were seen. Conclusions: Oral CPM showed a mild activity and good tolerability in advanced soft tissue and metastatic STS. It could be an appropriate solution as second line and further therapy and in unfit or elderly pts.


2014 ◽  
Vol 28 (4) ◽  
pp. 1048-1051 ◽  
Author(s):  
Raphael L.C. Araujo ◽  
Sébastien Gaujoux ◽  
Luiz Augusto Carneiro D'Albuquerque ◽  
Alain Sauvanet ◽  
Jacques Belghiti ◽  
...  

Author(s):  
Nizar Kardoun ◽  
Ahmed Bouzid_ahmed ◽  
Ayman Trigui ◽  
Haitham Rejab ◽  
Mohammed BenAmar ◽  
...  

Primary leimyosarcoma of the inferior vena cava is considered as rare vascular retroperitoneal sarcoma. Although radical resection with free margin is necessary, vascular strategies are very challenging.


2016 ◽  
Vol 51 (1) ◽  
pp. 56-64 ◽  
Author(s):  
Marko Novak ◽  
Andraz Perhavec ◽  
Katherine E. Maturen ◽  
Snezana Pavlovic Djokic ◽  
Simona Jereb ◽  
...  

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.


Medicine ◽  
2017 ◽  
Vol 96 (18) ◽  
pp. e6751 ◽  
Author(s):  
Abdulaziz Alghulayqah ◽  
Naif Alghasab ◽  
Tarik Amin ◽  
Nora Alkahtani ◽  
Rafif Farhat ◽  
...  

2021 ◽  
Vol 14 (1) ◽  
pp. e238437
Author(s):  
Sinduja Ramanan ◽  
Hemachandren Munuswamy ◽  
Vishnu Prasad Nelamangala Ramakrishnaiah ◽  
Pampa Ch Toi

Adrenal incidentalomas are incidentally detected adrenal lesions on imaging, which have a variety of differential diagnoses, the most common being a non-functioning adenoma. Surgical intervention for these lesions is needed when there is hypersecretion, for lesions larger than 4 cm and smaller lesions with suspicious characteristics. Here we present a young woman who was incidentally found to have a right suprarenal mass with loss of fat planes with the inferior vena cava (IVC). She underwent resection of the tumour along with the posterior wall of IVC, which was primarily repaired. Her postoperative biopsy was suggestive of leiomyosarcoma arising from the IVC. In the absence of distant metastasis, the sole prognostic factor for this tumour is achieving negative margins through radical resection of the tumour with IVC resection. Retroperitoneal leiomyosarcomas should be considered as a differential diagnosis for larger lesions, especially those more than 10 cm.


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