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Myoepithelial tumors represent a family of lesions with variable terminology, based on anatomic location: pleomorphic adenoma in the salivary gland (where myoepithelioma was first described), benign mixed tumor in the skin, and myoepithelial tumor or parachordoma in the soft tissues.

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  1. 1.

    EWSR1 (Ewing Sarcoma Breakpoint Region 1) encodes a multifunctional protein that is involved in various cellular processes, including gene expression, cell signaling, and RNA processing and transport. EWSR1 is a “promiscuous” gene because it can fuse with different partner genes in phenotypically similar neoplasms or with the same genes in morphologically and behaviorally different tumors. In fact, EWSR1-based chimeric genes can be found not only in Ewing and Ewing-like sarcomas but also in other tumors such as angiomatoid fibrous histiocytoma, clear cell sarcoma, low-grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma, hemangioma of bone, desmoplastic small round cell tumor, extraskeletal myxoid chondrosarcoma, myoepithelial tumor of soft tissue, and myxoid liposarcoma.

    It must be underscored that fluorescence in situ hybridization (FISH) analysis has a significant risk of false-negative results, making next-generation sequencing (NGS)-based diagnostic tools more sensitive for detecting EWSR1 rearrangements.

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Correspondence to Simone Mocellin .

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Mocellin, S. (2021). Myoepithelioma. In: Soft Tissue Tumors . Springer, Cham.

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-58709-3

  • Online ISBN: 978-3-030-58710-9

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