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      Primary Cystic Pleuropulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax


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          Primary pleuropulmonary synovial sarcomas are quite rare, representing 0.1–0.5% of all pulmonary malignancies. We report an entirely cystic monophasic synovial sarcoma in a 25-year-old male who presented with recurrent pneumothorax and no evidence of a mass lesion on imaging. The purpose of this case report is to increase awareness of neoplasms clinically presenting as a pneumothorax with no imagining evidence of a mass-forming lesion and emphasize the significance of fluorescent in situ hybridization testing in nontypical synovial sarcoma cases.

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          Most cited references15

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          Prognostic factors in advanced synovial sarcoma: an analysis of 104 patients treated at the Royal Marsden Hospital.

          Key prognostic factors at diagnosis of synovial sarcoma are well defined from the literature. There are few data regarding prognostic parameters in the setting of advanced disease. Our aim was to look specifically at a cohort of patients with advanced synovial sarcoma and to identify potential prognostic factors. One hundred and four patients with advanced synovial sarcoma were identified from the Royal Marsden Hospital's sarcoma database between 1978 and 2003. Patient data were analysed retrospectively. Most patients were aged between 20 and 50 years at diagnosis. Seventy-one patients were deceased at the time of analysis. Ninety-two patients received chemotherapy for management of advanced disease (most commonly doxorubicin + ifosfamide). Median survival following development of advanced disease was 22 months. Predictors of survival with advanced disease were age <35 years (P = 0.03) and response to first-line chemotherapy (P = 0.05). The response rate to doxorubicin plus ifosfamide was 58.6%, and this was superior to either agent when given singly. Metastasectomy was not associated with improved prognosis in this series. Synovial sarcoma is a chemosensitive soft tissue sarcoma. Compared with historical controls, survival with advanced disease seems to have improved over the years, possibly as a result of better use of chemotherapy. Age <35 years and response to first-line chemotherapy predict for improved survival with advanced disease.
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            From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation.

            Synovial sarcoma is the fourth most common type of soft-tissue sarcoma, accounting for 2.5%-10.5% of all primary soft-tissue malignancies worldwide. Synovial sarcoma most often affects the extremities (80%-95% of cases), particularly the knee in the popliteal fossa, of adolescents and young adults (15-40 years of age). Despite its name, the lesion does not commonly arise in an intraarticular location but usually occurs near joints. Histologic subtypes include monophasic, biphasic, and poorly differentiated; the cytogenetic aberration of the t(X;18) translocation is highly specific for synovial sarcoma. Although radiographic features of these tumors are not pathognomonic, findings of a soft-tissue mass, particularly if calcified (30%), near but not in a joint of a young patient, are very suggestive of the diagnosis. Cross-sectional imaging features are vital for staging tumor extent and planning surgical resection; they also frequently reveal suggestive appearances of multilobulation and marked heterogeneity (creating the "triple sign") with hemorrhage, fluid levels, and septa (creating the "bowl of grapes" sign). Two features associated with synovial sarcoma that may lead to an initial mistaken diagnosis of a benign indolent process are slow growth (average time to diagnosis, 2-4 years) and small size (< 5 cm at initial presentation); in addition, these lesions may demonstrate well-defined margins and homogeneous appearance on cross-sectional images. Synovial sarcoma is an intermediate- to high-grade lesion, and, despite initial aggressive wide surgical resection, local recurrence and metastatic disease are common and prognosis is guarded. Understanding and recognizing the spectrum of appearances of synovial sarcoma, which reflect the underlying pathologic characteristics, improve radiologic assessment and are important for optimal patient management. Copyright RSNA, 2006.
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              Prognostic factors in localized primary synovial sarcoma: a multicenter study of 128 adult patients.

              To identify most significant and therapeutically relevant prognostic factors in adults with localized primary synovial sarcomas (SS) and to confirm the usefulness of the French Federation of Cancer Centers (FNCLCC) grading system, the prognostic impact of which has been already proven in soft tissue sarcomas. Data on 128 patients with nonmetastatic SS collected from a cooperative database by the FNCLCC Sarcoma Group between 1980 and 1994 were studied retrospectively. Immunohistochemistry was performed at diagnosis in 77 cases (61%). The tumors were classified as biphasic (n = 45), monophasic fibrous (n = 72), and poorly differentiated (n = 10) subtypes. Histologic grade was determined according to the FNCLCC method, and vascular invasion was assessed in every case. The 5-year disease-specific survival (DSS) rate for this series of patients with localized SS was 62.9% (+/- 9.6% [SD]) with a median follow-up time of 37 months (range, 8 to 141 months). In multivariate analysis, the adverse risk factors associated with decreased DSS were International Union Against Cancer/American Joint Committee on Cancer stage III/IVA disease, male sex, and truncal tumor locations. For metastasis-free survival (MFS), disease stage III/IVA, tumor necrosis, and monophasic subtypes were the major factors associated with a less favorable prognosis. Separately, when not using disease stage, tumor necrosis, and mitotic activity, histologic grade became the most significant prognostic factor for both DSS and MFS. In addition, larger tumors and older patients become associated with a significantly worse prognosis. Independent adverse risk factors for local recurrence-free survival included histologic grade 3 and truncal tumor location. These data confirm that not all SS present the same severe outcome. High-risk patients identified on the basis of these parameters may qualify for an aggressive treatment approach.

                Author and article information

                Case Reports in Oncology
                S. Karger AG
                May – August 2017
                14 July 2017
                : 10
                : 2
                : 660-665
                [_a] aDepartment of Internal Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA
                [_b] bDepartment of Pathology, Cleveland Clinic Foundation, Cleveland, Ohio, USA
                [_c] cDepartment of Surgery, Division of Cardiothoracic Surgery, University of Utah School of Medicine, Salt Lake City, Utah, USA
                [_d] dDepartment of Pathology, University of Utah School of Medicine, Salt Lake City, Utah, USA
                Author notes
                *Eric D. Johnson, MD, Department of Internal Medicine, University of Utah School of Medicine, 1434 E 900 S, Salt Lake City, UT 84105 (USA), E-Mail Eric.Johnson@hsc.utah.edu
                478845 PMC5567016 Case Rep Oncol 2017;10:660–665
                © 2017 The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Figures: 4, Pages: 6
                Self URI (application/pdf): https://www.karger.com/Article/Pdf/478845
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                Self URI (journal page): https://www.karger.com/SubjectArea/Oncology
                Case Report

                Oncology & Radiotherapy,Pathology,Surgery,Obstetrics & Gynecology,Pharmacology & Pharmaceutical medicine,Hematology
                Primary pleuropulmonary synovial sarcoma,Pleuroplumonary synovial sarcoma,Cystic pleuropulmonary synovial sarcoma,Synovial sarcoma


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