Synovial Sarcoma

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Andrew E Horvai - One of the best experts on this subject based on the ideXlab platform.

  • diagnostic utility of sox10 to distinguish malignant peripheral nerve sheath tumor from Synovial Sarcoma including intraneural Synovial Sarcoma
    Modern Pathology, 2014
    Co-Authors: Yuna Kang, Melike Pekmezci, Andrew L Folpe, Ayca Ersen, Andrew E Horvai
    Abstract:

    Synovial Sarcoma and malignant peripheral nerve sheath tumor pose a significant diagnostic challenge given similar histomorphology. The distinction is further complicated by similar immunophenotype and especially by occasional Synovial Sarcomas that present as intraneural tumors. Although the presence of a t(X;18) rearrangement or expression of TLE1 can help confirm the diagnosis of Synovial Sarcoma, negative results for these tests are not diagnostic of malignant peripheral nerve sheath tumor. The SOX10 transcription factor, a putative marker of neural crest differentiation, may have diagnostic utility in this differential, but immunohistochemical data are limited. The goal of the present study was to determine the diagnostic utility of SOX10 to discriminate between Synovial Sarcoma and malignant peripheral nerve sheath tumor. Forty-eight cases of malignant peripheral nerve sheath tumor, all from patients with documented neurofibromatosis, and 97 cases of genetically confirmed Synovial Sarcoma, including 4 intraneural Synovial Sarcomas, were immunohistochemically stained for SOX10. The stain was scored for intensity and fraction of cells staining. Thirty-two of 48 malignant peripheral nerve sheath tumors (67%) were SOX10-positive. The majority of malignant peripheral nerve sheath tumors showed ≥2+ staining, but staining did not correlate with grade. By contrast, only 7/97 (7%) Synovial Sarcomas were SOX10-positive. Only three Synovial Sarcomas showed ≥2+ staining but, importantly, two of these were intraneural Synovial Sarcoma. Therefore, SOX10 is a specific (93%), albeit not very sensitive (67%), diagnostic marker to support a diagnosis of malignant peripheral nerve sheath tumor over Synovial Sarcoma. Furthermore, the stain needs to be interpreted with caution in intraneural tumors in order to avoid a potential diagnostic pitfall. It remains to be determined whether SOX10-positive cells in intraneural Synovial Sarcoma represent entrapped Schwann cells, Synovial Sarcoma cells or both.

Patrick A. Adegboyega - One of the best experts on this subject based on the ideXlab platform.

  • Primary cardiac Synovial Sarcoma.
    Archives of pathology & laboratory medicine, 2012
    Co-Authors: Tanya Varma, Patrick A. Adegboyega
    Abstract:

    Primary cardiac Synovial Sarcoma is an uncommon malignant neoplasm, with only a handful of cases reported in the English literature to date. Synovial Sarcomas have also been described at other unusual sites, such as the heart, pleuropulmonary region, kidney, prostate, liver, mediastinum, retroperitoneum, gastrointestinal tract, and peripheral nerve. For Synovial Sarcomas that arise at these unusual locations, definitive diagnosis is challenging and requires use of ancillary diagnostic procedures, such as immunohistochemistry, electron microscopy, and molecular genetic techniques, for confirmation of diagnosis. The nonrandom occurrence of t(X;18) has been found consistently in Synovial Sarcomas. It has also been found as a sole cytogenetic abnormality in some cases, suggesting it as a key molecular event in tumor development. This review highlights salient features of primary cardiac Synovial Sarcoma and the associated diagnostic challenges.

T Z Nursal - One of the best experts on this subject based on the ideXlab platform.

Samadarshi Datta - One of the best experts on this subject based on the ideXlab platform.

  • a primary Synovial Sarcoma of lung
    North American Journal of Medical Sciences, 2012
    Co-Authors: Anirban Sarkar, Amiya Kumar Dwari, Samadarshi Datta
    Abstract:

    Primary pulmonary Synovial Sarcoma is an extremely rare tumor. The diagnosis is established only after Sarcoma like primary lung malignancies and metastatic Sarcoma have been excluded. It has four subtypes: monophasic fibrous, monophasic epithelial, biphasic, and poorly differentiated subtypes, We report a case of a 55-year-old man, who complained of left-sided chest pain and shortness of breath, had a large heterogeneous mass, occupying most of left hemithorax, associated with ipsilateral pleural effusion, seen on contrast enhanced computed tomogram of thorax. Computed tomography guided tru-cut biopsy revealed spindle cell Sarcoma. On immunohistochemistry, tumor cells expressed epithelial membrane antigen, CD99, bcl-2 and Calponin and were immunonegative for cytokeratin. So, final diagnosis was primary pulmonary Synovial Sarcoma, Primary pulmonary Synovial Sarcoma is a rarely reported case of malignant neoplasm of lung. Histopathology, immunohistochemistry, and cytogenetics, if possible, are essential for confirmation of its diagnosis.

P Wang - One of the best experts on this subject based on the ideXlab platform.