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      Reasons why a second radiological opinion is advisable: a case report of a misreported crural synovial cell sarcoma

      case-report
      1 , 2 , , 1
      Journal of Surgical Case Reports
      Oxford University Press

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          Abstract

          Synovial sarcoma (SS) is a rare condition that accounts for 5–10% of all soft-tissue sarcomas (STS). SS locates most frequently near the joints, in particular at the lower extremities, but it can also occur in other locations. We report a case of a 42-year-old male complaining of a slow-growth mass on his right thigh, reported as a femoral nerve shwannoma on the basis of the preoperative radiological investigations, which revealed to be a monophasic SS on the histological examination. During the surgical procedure, the assistance of vascular surgeons was required to reconstruct the wall of the femoral vein underlying the tumor, that was pathologically thickened, and communicated with the tumoral capsule. Although extremely rare, SS should be considered in the differential diagnosis of peripheral nerve sheath tumors, in particular if next to a large vein at the lower extremities.

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          Most cited references22

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          Diagnosis and management of synovial sarcoma.

          Synovial sarcoma accounts about 9% of soft tissue sarcomas, most commonly develops in the extremity of young adults, is considered high grade and contains a characteristic translocation (X;18;p11;q11). While surgery and radiation therapy have achieved excellent local control, distant metastasis remains the principal problem limiting survival. Although ifosfamide based chemotherapy has been associated with an improved survival in patients with synovial sarcoma, the search for less toxic and more targeted systemic therapies is ongoing. (c) 2008 Wiley-Liss, Inc.
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            Synovial sarcoma: retrospective analysis of 250 patients treated at a single institution.

            The optimal treatment for synovial sarcoma remains controversial. Treatment, outcome, and prognostic factors in patients treated in a single institution were examined. Synovial sarcoma patients who underwent surgery at the Rizzoli Institute between 1976 and 2006 were identified and analyzed. Characteristics of the 250 patients (128 female; 122 male) included: median age, 37 years (range, 7-83 years); 177 (71%) with tumors in the lower extremity, 40 (16%) with tumors in the upper extremity, and 33 with tumors in the trunk (13%); primary lesion size >5 cm in 121 patients (55%); and 204 (82%) patients with localized disease and 46 (18%) with metastatic disease at the time of presentation. All patients with localized disease underwent surgery. Twenty-four percent of patients underwent amputation. Adequate surgical margins were achieved in 88% patients. In patients with localized disease, radiotherapy was administered to 103 (50%) patients, and chemotherapy to 98 (48%). With a median follow-up of 5.5 years (range, 1-30 years), the 5-year overall survival rate was 10% for patients with metastatic disease and 76% for patients with localized disease (P = .0001). The 5-year event-free survival was 58% in patients with localized disease. Multivariate analysis indicated that size, age, histologic subtype, and the use of radiotherapy were independent factors for event-free survival. In those patients with localized disease, a good rate of cure can be achieved. Age, size, histology, and use of radiotherapy influence prognosis, whereas to the authors' knowledge, the role of adjuvant chemotherapy remains unproven.
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              Synovial sarcoma of childhood and adolescence: a multicenter, multivariate analysis of outcome.

              To identify prognostic factors related to outcome in 219 children and adolescents with synovial sarcoma. We combined the experiences of the four following research groups: Cooperative Weichteilsarkomastudie Group, Germany (n = 95); St. Jude Children's Research Hospital, Memphis, TN (n = 49); Istituto Nazionale dei Tumori, Milan, Italy (n = 33); and The University of Texas M.D. Anderson Cancer Center, Houston, TX (n = 42). Kaplan-Meier and Cox proportional hazard analyses were performed. The median age at diagnosis was 13 years (range, 1 to 20 years), and the median follow-up was 6.6 years (range, 0.5 to 30.7 years). The estimated 5-year overall survival and event-free survival rates for the entire group were 80% +/- 3% (SE) and 72% +/- 3%, respectively. A previously unreported interaction between tumor size and invasiveness was observed that statistically significantly related to outcome. In multivarible analysis, patients with T1B and T2B disease (hazard ratio [HR] = 5.6, 95% confidence interval (CI), 1.9 to 16.2; and HR = 5.9, 95% CI, 2.1 to 16.4, respectively) or Intergroup Rhabdomyosarcoma Study (IRS) Clinical Group III and IV disease (HR = 2.7, 95% CI, 1.2 to 6.5; and HR = 14.1, 95% CI, 4.3 to 31.3, respectively) had poor overall survival. Treatment with radiotherapy was related to improved overall survival (HR = 0.4; 95% CI, 0.2 to 0.7). In IRS Group III patients, objective response to chemotherapy (18 of 30, 60%) correlated with improved survival. Clinical group, tumor size, and invasiveness are important prognostic factors. Multicenter randomized clinical trials are needed to determine both the effect of chemotherapy on survival and the necessity of local radiotherapy in patients with completely resected tumors.
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                Author and article information

                Journal
                J Surg Case Rep
                J Surg Case Rep
                jscr
                Journal of Surgical Case Reports
                Oxford University Press
                2042-8812
                March 2017
                09 March 2017
                09 March 2017
                : 2017
                : 3
                : rjx046
                Affiliations
                [1 ]Department of Neurosurgery, Anthea Hospital, Bari, Italy
                [2 ]Department of Neurosurgery, ‘Federico II’ University , Naples, Italy
                Author notes
                [* ]Corresponding address: Department of Neurosurgery, ‘Federico II’ University, via Sergio Pansini 5, 80131 Naples, Italy. Tel: +39-3398225931; Fax: +39-0817462579; E-mail: severinorocco@ 123456gmail.com
                Article
                rjx046
                10.1093/jscr/rjx046
                5400482
                a604a45c-292d-4f4a-89f9-8e4168d4e68e
                Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author 2017.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 14 December 2016
                : 01 March 2017
                Page count
                Pages: 4
                Categories
                Case Report

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