Synovial Sarcoma: A Complex Disease with Multifaceted Signaling and Epigenetic Landscapes

Marc El Beaino; Elie Rassy; Bana Hadid; Dejka M. Araujo; Nicholas Pavlidis; Patrick P. Lin

doi:10.1007/s11912-020-00985-w

Synovial Sarcoma: A Complex Disease with Multifaceted Signaling and Epigenetic Landscapes

Marc El Beaino, Elie Rassy, Bana Hadid, Dejka M. Araujo, Nicholas Pavlidis, Patrick P. Lin

Research output: Contribution to journal › Review article › peer-review

8 Scopus citations

Abstract

Purpose of Review: Aside from a characteristic SS18–SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of synovial sarcoma. In the following review, we explore the structural units of wild-type SS18 and SSX, particularly as they relate to the transcriptional alterations and cellular pathway changes imposed by SS18–SSX. Recent Findings: Native SS18 and SSX contribute recognizable domains to the SS18–SSX chimeric proteins, which inflict transcriptional and epigenetic changes through selective protein interactions involving the SWI/SNF and Polycomb chromatin remodeling complexes. Multiple oncogenic and developmental pathways become altered, collectively reprogramming the cellular origin of synovial sarcoma and promoting its malignant transformation. Summary: Synovial sarcoma is characterized by complex epigenetic and signaling landscapes. Identifying the operational pathways and concomitant genetic changes induced by SS18–SSX fusions could help develop tailored therapeutic strategies to ultimately improve disease control and patient survivorship.

Original language	English (US)
Article number	124
Journal	Current oncology reports
Volume	22
Issue number	12
DOIs	https://doi.org/10.1007/s11912-020-00985-w
State	Published - Dec 1 2020

Keywords

Polycomb complex
SS18–SSX
SWI/SNF complex
Synovial sarcoma
chromatin modulation
neoplastic pathways

ASJC Scopus subject areas

Oncology

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10.1007/s11912-020-00985-w

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title = "Synovial Sarcoma: A Complex Disease with Multifaceted Signaling and Epigenetic Landscapes",

abstract = "Purpose of Review: Aside from a characteristic SS18–SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of synovial sarcoma. In the following review, we explore the structural units of wild-type SS18 and SSX, particularly as they relate to the transcriptional alterations and cellular pathway changes imposed by SS18–SSX. Recent Findings: Native SS18 and SSX contribute recognizable domains to the SS18–SSX chimeric proteins, which inflict transcriptional and epigenetic changes through selective protein interactions involving the SWI/SNF and Polycomb chromatin remodeling complexes. Multiple oncogenic and developmental pathways become altered, collectively reprogramming the cellular origin of synovial sarcoma and promoting its malignant transformation. Summary: Synovial sarcoma is characterized by complex epigenetic and signaling landscapes. Identifying the operational pathways and concomitant genetic changes induced by SS18–SSX fusions could help develop tailored therapeutic strategies to ultimately improve disease control and patient survivorship.",

keywords = "Polycomb complex, SS18–SSX, SWI/SNF complex, Synovial sarcoma, chromatin modulation, neoplastic pathways",

author = "{El Beaino}, Marc and Elie Rassy and Bana Hadid and Araujo, {Dejka M.} and Nicholas Pavlidis and Lin, {Patrick P.}",

note = "Publisher Copyright: {\textcopyright} 2020, Springer Science+Business Media, LLC, part of Springer Nature.",

year = "2020",

month = dec,

day = "1",

doi = "10.1007/s11912-020-00985-w",

language = "English (US)",

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journal = "Current oncology reports",

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T2 - A Complex Disease with Multifaceted Signaling and Epigenetic Landscapes

AU - El Beaino, Marc

AU - Rassy, Elie

AU - Hadid, Bana

AU - Araujo, Dejka M.

AU - Pavlidis, Nicholas

AU - Lin, Patrick P.

PY - 2020/12/1

Y1 - 2020/12/1

N2 - Purpose of Review: Aside from a characteristic SS18–SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of synovial sarcoma. In the following review, we explore the structural units of wild-type SS18 and SSX, particularly as they relate to the transcriptional alterations and cellular pathway changes imposed by SS18–SSX. Recent Findings: Native SS18 and SSX contribute recognizable domains to the SS18–SSX chimeric proteins, which inflict transcriptional and epigenetic changes through selective protein interactions involving the SWI/SNF and Polycomb chromatin remodeling complexes. Multiple oncogenic and developmental pathways become altered, collectively reprogramming the cellular origin of synovial sarcoma and promoting its malignant transformation. Summary: Synovial sarcoma is characterized by complex epigenetic and signaling landscapes. Identifying the operational pathways and concomitant genetic changes induced by SS18–SSX fusions could help develop tailored therapeutic strategies to ultimately improve disease control and patient survivorship.

AB - Purpose of Review: Aside from a characteristic SS18–SSX translocation identified in almost all cases, no genetic anomalies have been reliably isolated yet to drive the pathogenesis of synovial sarcoma. In the following review, we explore the structural units of wild-type SS18 and SSX, particularly as they relate to the transcriptional alterations and cellular pathway changes imposed by SS18–SSX. Recent Findings: Native SS18 and SSX contribute recognizable domains to the SS18–SSX chimeric proteins, which inflict transcriptional and epigenetic changes through selective protein interactions involving the SWI/SNF and Polycomb chromatin remodeling complexes. Multiple oncogenic and developmental pathways become altered, collectively reprogramming the cellular origin of synovial sarcoma and promoting its malignant transformation. Summary: Synovial sarcoma is characterized by complex epigenetic and signaling landscapes. Identifying the operational pathways and concomitant genetic changes induced by SS18–SSX fusions could help develop tailored therapeutic strategies to ultimately improve disease control and patient survivorship.

KW - Polycomb complex

KW - SS18–SSX

KW - SWI/SNF complex

KW - Synovial sarcoma

KW - chromatin modulation

KW - neoplastic pathways

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ER -

Synovial Sarcoma: A Complex Disease with Multifaceted Signaling and Epigenetic Landscapes

Abstract

Keywords

ASJC Scopus subject areas

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