Synovial sarcoma: when epigenetic changes dictate tumour development
Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation that results in the formation and expression of the fusion gene SS18-SSX, which underlies its pathogenesis. Although SS18-SSX pro...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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SMW supporting association (Trägerverein Swiss Medical Weekly SMW)
2018-12-01
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| Series: | Swiss Medical Weekly |
| Subjects: | |
| Online Access: | https://www.smw.ch/index.php/smw/article/view/2544 |
| _version_ | 1797979660896698368 |
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| author | Nicolo Riggi Luisa Cironi Ivan Stamenkovic |
| author_facet | Nicolo Riggi Luisa Cironi Ivan Stamenkovic |
| author_sort | Nicolo Riggi |
| collection | DOAJ |
| description |
Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation that results in the formation and expression of the fusion gene SS18-SSX, which underlies its pathogenesis. Although SS18-SSX provides a potentially unique therapeutic target, all attempts to neutralise it have been unsuccessful thus far. When complete surgical removal of the tumour fails, therapy is limited to largely ineffective cytotoxic drug regimens. Nevertheless, recent discoveries about the mechanisms of SS18-SSX protein function have provided insight into potential alternative therapeutic strategies. SS18-SSX displays oncogenic activity through protein-protein interactions and participation in chromatin remodelling complexes. This review summarises our current understanding of the function of SS18-SSX and the mechanisms by which it alters the epigenetic landscape of permissive cells to induce transformation and the subsequent development of synovial sarcoma.
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| first_indexed | 2024-04-11T05:42:25Z |
| format | Article |
| id | doaj.art-3fcfed830590426b8468c1576b642893 |
| institution | Directory Open Access Journal |
| issn | 1424-3997 |
| language | English |
| last_indexed | 2024-04-11T05:42:25Z |
| publishDate | 2018-12-01 |
| publisher | SMW supporting association (Trägerverein Swiss Medical Weekly SMW) |
| record_format | Article |
| series | Swiss Medical Weekly |
| spelling | doaj.art-3fcfed830590426b8468c1576b6428932022-12-22T04:42:27ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972018-12-01148474810.4414/smw.2018.14667Synovial sarcoma: when epigenetic changes dictate tumour developmentNicolo RiggiLuisa CironiIvan Stamenkovic Synovial sarcoma is a highly aggressive soft tissue malignancy that often affects adolescents and young adults. It is associated with a unique chromosomal translocation that results in the formation and expression of the fusion gene SS18-SSX, which underlies its pathogenesis. Although SS18-SSX provides a potentially unique therapeutic target, all attempts to neutralise it have been unsuccessful thus far. When complete surgical removal of the tumour fails, therapy is limited to largely ineffective cytotoxic drug regimens. Nevertheless, recent discoveries about the mechanisms of SS18-SSX protein function have provided insight into potential alternative therapeutic strategies. SS18-SSX displays oncogenic activity through protein-protein interactions and participation in chromatin remodelling complexes. This review summarises our current understanding of the function of SS18-SSX and the mechanisms by which it alters the epigenetic landscape of permissive cells to induce transformation and the subsequent development of synovial sarcoma. https://www.smw.ch/index.php/smw/article/view/2544ChromatinEpigeneticspathogenesisSS18-SSXsynovial sarcoma |
| spellingShingle | Nicolo Riggi Luisa Cironi Ivan Stamenkovic Synovial sarcoma: when epigenetic changes dictate tumour development Swiss Medical Weekly Chromatin Epigenetics pathogenesis SS18-SSX synovial sarcoma |
| title | Synovial sarcoma: when epigenetic changes dictate tumour development |
| title_full | Synovial sarcoma: when epigenetic changes dictate tumour development |
| title_fullStr | Synovial sarcoma: when epigenetic changes dictate tumour development |
| title_full_unstemmed | Synovial sarcoma: when epigenetic changes dictate tumour development |
| title_short | Synovial sarcoma: when epigenetic changes dictate tumour development |
| title_sort | synovial sarcoma when epigenetic changes dictate tumour development |
| topic | Chromatin Epigenetics pathogenesis SS18-SSX synovial sarcoma |
| url | https://www.smw.ch/index.php/smw/article/view/2544 |
| work_keys_str_mv | AT nicoloriggi synovialsarcomawhenepigeneticchangesdictatetumourdevelopment AT luisacironi synovialsarcomawhenepigeneticchangesdictatetumourdevelopment AT ivanstamenkovic synovialsarcomawhenepigeneticchangesdictatetumourdevelopment |