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01-12-2020 | Solid Tumor | Editorial

Targeting epigenetics in sarcomas through EZH2 inhibition

Author: Antoine Italiano

Published in: Journal of Hematology & Oncology | Issue 1/2020

Abstract

Soft-tissue sarcomas represent a heterogeneous group of diseases with distinct genetic and clinical features accounting for up to 1% of cancer in adults and 15% of cancer in children. Epithelioid sarcoma is an extremely rare and aggressive tumor affecting young adults that is characterized by loss of INI1 expression. INI1 (SMARCB1, SNF5, BAF47) is a subunit of the SWI/SNF chromatin remodeling complex that opposes the enzymatic function of EZH2. When INI1 loses its regulatory function, EZH2 activity is de-regulated, allowing EZH2 to play a driving, oncogenic role. Tazemetostat, a specific EZH2 inhibitor, has just been approved for patients with advanced epithelioid sarcoma and represents a new therapeutic option in this devastating disease.

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Title: Targeting epigenetics in sarcomas through EZH2 inhibition
Author: Antoine Italiano
Publication date: 01-12-2020
Publisher: BioMed Central
Keywords: Solid Tumor
Soft Tissue Sarcoma
Sarcoma
Epigenetics
Published in: Journal of Hematology & Oncology / Issue 1/2020
Electronic ISSN: 1756-8722
DOI: https://doi.org/10.1186/s13045-020-00868-4

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