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SS18-SSX Fusion Protein Expression

MedGen UID:
313685
•Concept ID:
C1709998
•
Cell or Molecular Dysfunction
Synonyms: SS18/SSX Fusion Protein Expression; SSXT-SSX Fusion Protein Expression; SYT-SSX Fusion Protein Expression

Definition

The expression of fusion proteins that result from a chromosomal aberration, t(X;18)(p11.2;q11.2), which fuses the first 10 exons of the SS18 gene with exon six of one of three SSX genes (SSX1, SSX2 or SSX4) and is associated with synovial sarcoma. [from NCI]

Professional guidelines

PubMed

Utility of Immunohistochemistry With Antibodies to SS18-SSX Chimeric Proteins and C-Terminus of SSX Protein for Synovial Sarcoma Differential Diagnosis.
Lasota J, Chłopek M, Kaczorowski M, Natálie K, Ryś J, Kopczyński J, Sulaieva O, Michal M, Kruczak A, Harazin-Lechowska A, Szczepaniak M, Koshyk O, Hałoń A, Czapiewski P, Abdullaev Z, Kowalik A, Aldape KD, Michal M, Miettinen M
Am J Surg Pathol 2024 Jan 1;48(1):97-105. Epub 2023 Oct 27 doi: 10.1097/PAS.0000000000002144. PMID: 37899499

Recent clinical studies

Etiology

Combinatorial strategies to potentiate the efficacy of HDAC inhibitors in fusion-positive sarcomas.
Lanzi C, Cassinelli G
Biochem Pharmacol 2022 Apr;198:114944. Epub 2022 Feb 7 doi: 10.1016/j.bcp.2022.114944. PMID: 35152144
Expression of TLE1, INI1, β-catenin, Claudin1, CK7, CK19, SS18 and calponin in synovial sarcoma.
Madakshira MG, Radotra BD, Kaman L, Saikia UN
Indian J Pathol Microbiol 2021 Oct-Dec;64(4):707-716. doi: 10.4103/IJPM.IJPM_1137_20. PMID: 34673590
Histology and grading are important prognostic factors in synovial sarcoma.
Bianchi G, Sambri A, Righi A, Dei Tos AP, Picci P, Donati D
Eur J Surg Oncol 2017 Sep;43(9):1733-1739. Epub 2017 May 25 doi: 10.1016/j.ejso.2017.05.020. PMID: 28579008
Histone deacetylase inhibitors reverse SS18-SSX-mediated polycomb silencing of the tumor suppressor early growth response 1 in synovial sarcoma.
Lubieniecka JM, de Bruijn DR, Su L, van Dijk AH, Subramanian S, van de Rijn M, Poulin N, van Kessel AG, Nielsen TO
Cancer Res 2008 Jun 1;68(11):4303-10. doi: 10.1158/0008-5472.CAN-08-0092. PMID: 18519690
The (epi)genetics of human synovial sarcoma.
de Bruijn DR, Nap JP, van Kessel AG
Genes Chromosomes Cancer 2007 Feb;46(2):107-17. doi: 10.1002/gcc.20399. PMID: 17117414

Diagnosis

Utility of Immunohistochemistry With Antibodies to SS18-SSX Chimeric Proteins and C-Terminus of SSX Protein for Synovial Sarcoma Differential Diagnosis.
Lasota J, Chłopek M, Kaczorowski M, Natálie K, Ryś J, Kopczyński J, Sulaieva O, Michal M, Kruczak A, Harazin-Lechowska A, Szczepaniak M, Koshyk O, Hałoń A, Czapiewski P, Abdullaev Z, Kowalik A, Aldape KD, Michal M, Miettinen M
Am J Surg Pathol 2024 Jan 1;48(1):97-105. Epub 2023 Oct 27 doi: 10.1097/PAS.0000000000002144. PMID: 37899499
SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients.
Challa B, Mohanty SK, Jha S, Sampat NY, Sardana R, Lobo A, Sharma S, Arora S, Rath D, Munjal G, Pattnaik N, Jain D, Jain E, Dewan A, Dixit M, Malik V, Shinde S, Balzer BL, Parwani A
Int J Surg Pathol 2023 Oct;31(7):1232-1243. Epub 2023 Jan 2 doi: 10.1177/10668969221143481. PMID: 36591871
Expanding the Use of an SS18-SSX Antibody for Molecular Assays in Synovial Sarcoma.
Raquib AR, Hofvander J, Ta M, Nielsen TO
Appl Immunohistochem Mol Morphol 2022 Sep 1;30(8):531-539. Epub 2022 Jul 27 doi: 10.1097/PAI.0000000000001049. PMID: 35880992Free PMC Article
Identification of novel SSX1 fusions in synovial sarcoma.
Yoshida A, Arai Y, Satomi K, Kubo T, Ryo E, Matsushita Y, Hama N, Sudo K, Komiyama M, Yatabe Y, Shibata T, Ichikawa H, Ichimura K, Kawai A, Mori T
Mod Pathol 2022 Feb;35(2):228-239. Epub 2021 Sep 9 doi: 10.1038/s41379-021-00910-x. PMID: 34504309
Expression of TLE1, INI1, β-catenin, Claudin1, CK7, CK19, SS18 and calponin in synovial sarcoma.
Madakshira MG, Radotra BD, Kaman L, Saikia UN
Indian J Pathol Microbiol 2021 Oct-Dec;64(4):707-716. doi: 10.4103/IJPM.IJPM_1137_20. PMID: 34673590

Therapy

Combinatorial strategies to potentiate the efficacy of HDAC inhibitors in fusion-positive sarcomas.
Lanzi C, Cassinelli G
Biochem Pharmacol 2022 Apr;198:114944. Epub 2022 Feb 7 doi: 10.1016/j.bcp.2022.114944. PMID: 35152144
ATR Is a Therapeutic Target in Synovial Sarcoma.
Jones SE, Fleuren EDG, Frankum J, Konde A, Williamson CT, Krastev DB, Pemberton HN, Campbell J, Gulati A, Elliott R, Menon M, Selfe JL, Brough R, Pettitt SJ, Niedzwiedz W, van der Graaf WTA, Shipley J, Ashworth A, Lord CJ
Cancer Res 2017 Dec 15;77(24):7014-7026. Epub 2017 Oct 16 doi: 10.1158/0008-5472.CAN-17-2056. PMID: 29038346Free PMC Article

Prognosis

Histology and grading are important prognostic factors in synovial sarcoma.
Bianchi G, Sambri A, Righi A, Dei Tos AP, Picci P, Donati D
Eur J Surg Oncol 2017 Sep;43(9):1733-1739. Epub 2017 May 25 doi: 10.1016/j.ejso.2017.05.020. PMID: 28579008
Synovial Sarcoma With Myoid Differentiation.
Qassid O, Ali A, Thway K
Int J Surg Pathol 2016 Sep;24(6):525-7. Epub 2016 Apr 22 doi: 10.1177/1066896916645187. PMID: 27106779
SS18-SSX-regulated miR-17 promotes tumor growth of synovial sarcoma by inhibiting p21WAF1/CIP1.
Minami Y, Kohsaka S, Tsuda M, Yachi K, Hatori N, Tanino M, Kimura T, Nishihara H, Minami A, Iwasaki N, Tanaka S
Cancer Sci 2014 Sep;105(9):1152-9. Epub 2014 Sep 3 doi: 10.1111/cas.12479. PMID: 24989082Free PMC Article
Truncated SSX protein suppresses synovial sarcoma cell proliferation by inhibiting the localization of SS18-SSX fusion protein.
Yoneda Y, Ito S, Kunisada T, Morimoto Y, Kanzaki H, Yoshida A, Shimizu K, Ozaki T, Ouchida M
PLoS One 2013;8(10):e77564. Epub 2013 Oct 9 doi: 10.1371/journal.pone.0077564. PMID: 24130893Free PMC Article
The (epi)genetics of human synovial sarcoma.
de Bruijn DR, Nap JP, van Kessel AG
Genes Chromosomes Cancer 2007 Feb;46(2):107-17. doi: 10.1002/gcc.20399. PMID: 17117414

Clinical prediction guides

SS18-SSX Expression in a Contemporary Cohort of Primary Renal Synovial Sarcoma: A Multi-Institutional Experience of Fourteen Patients.
Challa B, Mohanty SK, Jha S, Sampat NY, Sardana R, Lobo A, Sharma S, Arora S, Rath D, Munjal G, Pattnaik N, Jain D, Jain E, Dewan A, Dixit M, Malik V, Shinde S, Balzer BL, Parwani A
Int J Surg Pathol 2023 Oct;31(7):1232-1243. Epub 2023 Jan 2 doi: 10.1177/10668969221143481. PMID: 36591871
Frequent TRPS1 expression in synovial sarcoma is associated with SS18-SSX fusion oncoprotein activity.
Cloutier JM, Ingram DR, Wani K, Lazar AJ, Wang WL
Hum Pathol 2022 Dec;130:88-94. Epub 2022 Sep 24 doi: 10.1016/j.humpath.2022.09.006. PMID: 36162599
A Role for SMARCB1 in Synovial Sarcomagenesis Reveals That SS18-SSX Induces Canonical BAF Destruction.
Li J, Mulvihill TS, Li L, Barrott JJ, Nelson ML, Wagner L, Lock IC, Pozner A, Lambert SL, Ozenberger BB, Ward MB, Grossmann AH, Liu T, Banito A, Cairns BR, Jones KB
Cancer Discov 2021 Oct;11(10):2620-2637. Epub 2021 Jun 2 doi: 10.1158/2159-8290.CD-20-1219. PMID: 34078620Free PMC Article
Paracrine osteoprotegerin and β-catenin stabilization support synovial sarcomagenesis in periosteal cells.
Barrott JJ, Illum BE, Jin H, Hedberg ML, Wang Y, Grossmann A, Haldar M, Capecchi MR, Jones KB
J Clin Invest 2018 Jan 2;128(1):207-218. Epub 2017 Nov 20 doi: 10.1172/JCI94955. PMID: 29202462Free PMC Article
Truncated SSX protein suppresses synovial sarcoma cell proliferation by inhibiting the localization of SS18-SSX fusion protein.
Yoneda Y, Ito S, Kunisada T, Morimoto Y, Kanzaki H, Yoshida A, Shimizu K, Ozaki T, Ouchida M
PLoS One 2013;8(10):e77564. Epub 2013 Oct 9 doi: 10.1371/journal.pone.0077564. PMID: 24130893Free PMC Article

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