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Malignant Peripheral Nerve Sheath Tumor

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Soft Tissue Tumors

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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a malignant neoplasm arising from the Schwann cells of a peripheral nerve. It is also referred to as malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma.

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Notes

  1. 1.

    BET proteins: Epigenetic writers, erasers, and readers are three categories of chromatin regulators for which small-molecule inhibitors have been developed. BET proteins are a family of epigenetic reader: of these, BET bromodomain protein BRD4 has recently emerged as an important chromatin-regulatory protein across multiple cancer types. BET proteins are characterized by two tandem bromodomains (B), which bind acetylated histones to support the recruitment of transcription elongation factor machinery to open chromatin regions, and an extra-terminal domain (ET) that mediates protein-protein interactions. BET proteins often interact with and support the activity of key transcription factors such as c-Myc, c-Jun, and p53. Members of the BET protein family, including BRD4, BRD3, and BRD2, can be potently inhibited pharmacologically with pan-BET bromodomain inhibitors (BET inhibitors) including JQ1, I-BET151, CPI-203, or OTX-015, which competitively bind both bromodomains, perturbing BET protein association with histones and subsequent regulation of gene expression.

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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). Malignant Peripheral Nerve Sheath Tumor. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_167

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  • DOI: https://doi.org/10.1007/978-3-030-58710-9_167

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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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