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      Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation—Part 1, Lymphatic Malformation Mimickers

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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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            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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              Hemangiomas and Vascular Malformations in Infants and Children

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                Author and article information

                Journal
                American Journal of Roentgenology
                American Journal of Roentgenology
                American Roentgen Ray Society
                0361-803X
                1546-3141
                May 2016
                May 2016
                : 206
                : 5
                : 940-951
                Article
                10.2214/AJR.15.15793
                26999565
                93b4419d-4941-41a8-a458-19cc433e64f9
                © 2016
                History

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