Rhabdoid Variant of Myoepithelial Carcinoma,with EWSR1 Rearrangement: Expanding the Spectrum of EWSR1-Rearranged Myoepithelial Tumors |
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Authors: | Khin Thway Nick Bown Aisha Miah Rob Turner Cyril Fisher |
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Affiliation: | .Sarcoma Unit, Royal Marsden Hospital, London, UK ;.Northern Genetics Service, Newcastle upon Tyne, UK ;.St James’ Institute for Oncology, Leeds, UK ;.Department of Histopathology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK |
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Abstract: | Myoepithelial and mixed tumors represent a heterogeneous group of neoplasms for which classification is incomplete and continues to evolve. Those arising in the soft tissues appear to represent subgroups that are genetically distinct from those that occur within salivary glands. We describe a case of soft tissue myoepithelial carcinoma with rhabdoid morphology, which presented as an enlarging neck mass in a 40 year old male, and in which EWSR1 rearrangement was demonstrated by fluorescence in situ hybridization. This neoplasm showed diffuse INI1 loss, making distinction from other INI1-negative rhabdoid tumors difficult. This expands the range of reported histologic features of EWSR1-rearranged myoepithelial neoplasms, and highlights the significant morphologic and immunohistochemical overlap between this and other INI1-negative malignant rhabdoid neoplasms. |
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Keywords: | EWSR1 Malignant rhabdoid tumor Myoepithelial carcinoma Translocation |
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