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Epithelioid Sarcoma

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Soft Tissue Tumors

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Abstract

Epithelioid sarcoma is a malignant tumor of uncertain differentiation. As described below, two variants are recognized:

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Notes

  1. 1.

    EMA: epithelial membrane antigen.

  2. 2.

    SMARCB1: SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily B, member 1 (also known as INI1) encodes a protein which is part of the BAF (hSWI/SNF) complex that relieves repressive chromatin structures, allowing the transcriptional machinery to access its targets more effectively. This ATP-dependent chromatin-remodeling complex plays an important role in different cell activities including cell differentiation; in particular, regulation of the stem cell-associated programme, which is maintained by the repressive effect of the EZH2-dependent PRC2 (polycomb repressive complex 2), is disrupted by SMARCB1. Overall, SMARCB1 has been found to act as a tumor suppressor, and its mutations have been associated with malignancies including sarcomas.

  3. 3.

    FNCLCC: Fédération Nationale des Centres de Lutte Contre Le Cancer.

  4. 4.

    EZH2: Enhancer of zeste 2 polycomb repressive complex 2 subunit encodes a member of the Polycomb group (PcG) family. In particular, it encodes the catalytic subunit of the PRC2/EED-EZH2 complex, which methylates Lys-9 (H3K9me) and Lys-27 (H3K27me) of histone H3, leading to transcriptional repression of the affected target gene. The dysregulation of this methylation is believed to be critical in the development of cancer.

  5. 5.

    FDA: Food and Drug Administration.

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Authors and Affiliations

  1. Department of Surgery Oncology and Gastroenterology, University of Padova, Padova, Italy

    Simone Mocellin

  2. Melanoma and Soft Tissue Sarcoma Unit, Istituto Oncologico Veneto (IOV-IRCCS) of Padova, Padova, Italy

    Simone Mocellin

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  1. Simone Mocellin
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Correspondence to Simone Mocellin .

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Mocellin, S. (2021). Epithelioid Sarcoma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_86

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  • DOI: https://doi.org/10.1007/978-3-030-58710-9_86

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-58709-3

  • Online ISBN: 978-3-030-58710-9

  • eBook Packages: MedicineMedicine (R0)

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