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Rhabdoid Variant of Myoepithelial Carcinoma, with EWSR1 Rearrangement: Expanding the Spectrum of EWSR1-Rearranged Myoepithelial Tumors

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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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Acknowledgments

The authors are grateful to Mr. John Swansbury and Dr. Toon Min (Haematology Cytogenetics, Royal Marsden Hospital and The Institute of Cancer Research) for FISH studies. The authors also acknowledge support from the NIHR RM/ICR Biomedical Research Centre.

Conflict of interest

The authors have no conflicts of interest or funding to disclose.

Author information

Authors and Affiliations

  1. Sarcoma Unit, Royal Marsden Hospital, London, UK

    Khin Thway, Aisha Miah & Cyril Fisher

  2. Northern Genetics Service, Newcastle upon Tyne, UK

    Nick Bown

  3. St James’ Institute for Oncology, Leeds, UK

    Rob Turner

  4. Department of Histopathology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ, UK

    Khin Thway

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  1. Khin Thway
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Correspondence to Khin Thway.

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Thway, K., Bown, N., Miah, A. et al. Rhabdoid Variant of Myoepithelial Carcinoma, with EWSR1 Rearrangement: Expanding the Spectrum of EWSR1-Rearranged Myoepithelial Tumors. Head and Neck Pathol 9, 273–279 (2015). https://doi.org/10.1007/s12105-014-0556-2

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  • DOI: https://doi.org/10.1007/s12105-014-0556-2

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