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      Renal Leiomyosarcoma

      case-report

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          Abstract

          Leiomyosarcoma (LMS) is a rare malignant tumor of smooth muscle origin that generally stems from soft tissues and uterine tissue. Although, a small percentage of these may originate from the smooth muscle or vessel walls, most of which are of venous origin. Renal leiomyosarcomas may arise from the smooth muscle fibers of renal pelvis, renal capsule or renal vessels, last one is the most frequent. We report a case of renal LMS that could be originated in the renal capsule.

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          Phase III randomised study to evaluate the role of adjuvant pelvic radiotherapy in the treatment of uterine sarcomas stages I and II: an European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group Study (protocol 55874).

          N S Reed, C Mangioni, H Malmström … (2008)
          The management of uterine sarcomas continues to present many difficulties. Primary surgery is the optimal treatment but the role of post-operative radiation remains uncertain. In the mid-1980s, the European Organisation for Research and Treatment of Cancer Gynaecological Cancer Group Study proposed a trial to evaluate adjuvant radiotherapy, as previous non-randomised studies had suggested a survival advantage and improved local control when post-operative radiation was administered. The study opened in 1987 taking 13 years to accrue 224 patients. All uterine sarcoma subtypes were permitted. Patients were required to have undergone as a minimum, TAH and BSO and wahsings (166 patients) but nodal sampling was optional. There were 103 leiomyosarcomas (LMS), 91 carcinosarcomas (CS) and 28 endometrial stromal sarcomas (ESS). Patients were randomised to either observation or pelvic radiation, 51 Gy in 28 fractions over 5 weeks. Hundred and twelve were recruited to each arm. The initial analysis has shown a reduction in local relapse (14 versus 24, p=0.004) but no effect on either OS or PFS. No unexpected toxicity was seen in the radiation arm. No difference in either overall or disease-free survival was demonstrated but there is an increased local control for the CS patients receiving radiation but without any benefit for LMS. Prognostic factor analysis shows that stage, age and histological subtype were important predictors of behaviour which may explain differences between CS and LMS. CS appears to show more kinship to poorly differentiated endometrial carcinomas in behaviour. LMS did not show the same benefit from radiation. These results will help shape future management and clinical trials in uterine sarcomas.
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            Phase II study of sunitinib malate, a multitargeted tyrosine kinase inhibitor in patients with relapsed or refractory soft tissue sarcomas. Focus on three prevalent histologies: leiomyosarcoma, liposarcoma and malignant fibrous histiocytoma.

            Scott Antonia, Michael Schell, Christopher Garrett … (2011)
            Soft tissue sarcomas (STS) represent a diverse group of histologic subtypes with targetable molecular alterations, often treated as a single disease. Sunitinib malate is a multitargeted receptor tyrosine kinase inhibitor active in other solid tumors carrying similar alterations (i.e., imatinib mesylate-refractory gastrointestinal stromal tumors). This single-institution phase II study investigated the safety and efficacy of sunitinib malate in three common STS subtypes. Patients with documented unresectable or metastatic STS (liposarcoma, leiomyosarcoma and malignant fibrous histiocytoma [MFH]), measurable disease, and 3 or less prior lines of therapy were eligible. Treatment consisted of sunitinib malate, 50 mg daily, for 4 weeks every 6 weeks. Forty-eight patients were enrolled, and 35% were heavily pretreated (≥ 2 prior lines of chemotherapy). The safety profile resembled previously known sunitinib malate toxicities. Median progression-free and overall survivals for liposarcoma, leiomyosarcoma, and MFH were 3.9 and 18.6, 4.2 and 10.1 and 2.5 and 13.6 months, respectively. The 3-month progression-free rates in the untreated and pretreated (chemotherapy) patients with liposarcoma, leiomyosarcoma and MFH were 75% and 69.2%, 60%, and 62.5% and 25% and 44.4%, respectively. With the caveats that a minority of patients with potentially indolent or low-grade disease could have been included and the small numbers, a 3-month progression-free rate of >40% suggests activity for sunitinib malate at least in liposarcomas and leiomyosarcomas. Thus, we believe that further investigation in these susceptible STS subtypes is warranted. Copyright © 2010 UICC.
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              Retroperitoneal sarcomas: Combined-modality treatment approaches.

              Mahendra P Raut, Peter Pisters (2006)
              Retroperitoneal sarcomas (RPS) are rare tumors, accounting for approximately 15% of soft tissue sarcomas. Surgical resection of localized tumors with gross and microscopically negative margins remains the standard of care. However, because RPS are frequently large and locally advanced, resections are often incomplete, resulting in local recurrence. Investigators are evaluating combined-modality therapies to improve local control and disease-specific survival. This review outlines current concepts and evolving treatment strategies in the diagnosis, staging, and management of RPS. Copyright 2006 Wiley-Liss, Inc.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Rare Tumors
                Journal ID (iso-abbrev): Rare Tumors
                Journal ID (publisher-id): RT
                Title: Rare Tumors
                Publisher: PAGEPress Publications, Pavia, Italy
                ISSN (Print): 2036-3605
                ISSN (Electronic): 2036-3613
                Publication date (Electronic): 04 September 2013
                Publication date Collection: 01 July 2013
                Volume: 5
                Issue: 3
                Electronic Location Identifier: e42
                Affiliations
                [1 ]Department of Urology, Hospital de Jerez, Cádiz University
                [2 ]Department of Urology, Hospital Puerta del Mar, Cádiz University
                [3 ]Department of Pathological Anatomy, Hospital de Jerez, Cádiz University , Spain
                Author notes
                Department of Urology, Hospital de Jerez, Cádiz University, Carretera de Circunvalación, N-IV Km 638. 11407 Jerez de la Frontera, Cádiz, Spain. Tel. +34.956.032135 - Fax: +34.956.032135 E-mail: otragadita@ 123456gmail.com
                Contributions: PB, manuscript writing and reviewing, and references searc, picture of surgical specimen and tomography; MJL, manuscript writing and reviewing; JLRC, microscopic picture.
                Conflict of interests: the authors declare no potential conflict of interest.
                Article
                DOI: 10.4081/rt.2013.e42
                PMC ID: 3804817
                PubMed ID: 24179654
                SO-VID: 5d7776cc-7a48-474f-9002-bbdeb8ca446b
                Copyright © ©Copyright P. Beardo et al.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 4 February 2013
                Date revision received : 27 May 2013
                Date accepted : 29 May 2013
                Page count
                Figures: 3, Tables: 0, Equations: 0, References: 12, Pages: 2
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: leiomyosarcoma,renal capsule,sarcoma
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: leiomyosarcoma, renal capsule, sarcoma

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