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Alveolar Soft Part Sarcoma

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Soft Tissue Tumors

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Abstract

Alveolar soft part sarcoma is a malignant tumor of uncertain differentiation. It was previously referred to as malignant myoblastoma, granular cell myoblastoma, and malignant granular cell myoblastoma.

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Notes

  1. 1.

    PAS: periodic acid Schiff.

  2. 2.

    The ASPSCR1-TFE3 fusion protein localizes to the nucleus where it acts as a transcription factor causing activation of the MET signaling pathway (which might make ASPS sensitive to MET inhibitors). TFE3 belongs to the microphthalmia family of bHLH-LZ transcription factors (MiT/TFE) which is composed of four members: MITF, TFEB, TFE3, and TFEC; neoplasms with alterations in these genes are also called MiT family tumors.

  3. 3.

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

  4. 4.

    EpSSG: European paediatric Soft tissue sarcoma Study Group.

  5. 5.

    SEER: Surveillance Epidemiology and End Results program.

  6. 6.

    Sunitinib: tyrosine kinase inhibitor targeting the following: PDGFR, VEGFR, KIT, FLT3, CSF1R, RET.

  7. 7.

    Pazopanib: tyrosine kinase inhibitor targeting the following: VEGFR, PDGFR, KIT.

  8. 8.

    Cediranib: tyrosine kinase inhibitor targeting the following: VEGFR.

  9. 9.

    NCCN: National Comprehensive Cancer Network.

  10. 10.

    ESMO: European Society for Medical Oncology.

  11. 11.

    Axitinib: tyrosine kinase inhibitor targeting the following: VEGFR.

  12. 12.

    Pembrolizumab: monoclonal antibody blocking the following: PD1.

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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). Alveolar Soft Part Sarcoma. In: Soft Tissue Tumors . Springer, Cham. https://doi.org/10.1007/978-3-030-58710-9_14

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  • DOI: https://doi.org/10.1007/978-3-030-58710-9_14

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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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