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EWSR1-ATF1 Fusion Protein Expression

MedGen UID:
268443
•Concept ID:
C1512145
•
Cell or Molecular Dysfunction
Synonyms: EWS-ATF1 Fusion Protein Expression; EWS/ATF1 Fusion Protein Expression; EWSR1/ATF1 Fusion Protein Expression

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEWSR1-ATF1 Fusion Protein Expression

Recent clinical studies

Etiology

Re-evaluating a historic cohort of sinonasal and skull base mucoepidermoid carcinoma: an institutional experience.
Hu C, Lin L, Ye M, Liu Y, Huang Q, Yuan C, Sun J, Sun H
Diagn Pathol 2024 Mar 1;19(1):46. doi: 10.1186/s13000-024-01466-5. PMID: 38429827Free PMC Article
Inflammatory and Nested Testicular Sex Cord Tumor: A Novel Neoplasm With Aggressive Clinical Behavior and Frequent EWSR1::ATF1 Gene Fusions.
Acosta AM, Bridge JA, Dal Cin PS, Sholl LM, Cornejo KM, Fletcher CDM, Ulbright TM
Am J Surg Pathol 2023 Apr 1;47(4):504-517. Epub 2023 Feb 15 doi: 10.1097/PAS.0000000000002022. PMID: 36791251
Intracranial mesenchymal tumors with FET-CREB fusion are composed of at least two epigenetic subgroups distinct from meningioma and extracranial sarcomas.
Sloan EA, Gupta R, Koelsche C, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Reddy A, Mueller S, Cho SJ, Horvai AE, Lee JC, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, von Deimling A, Chang SM, Berger MS, Solomon DA
Brain Pathol 2022 Jul;32(4):e13037. Epub 2021 Nov 25 doi: 10.1111/bpa.13037. PMID: 34821426Free PMC Article
Intracranial mesenchymal tumor with FET-CREB fusion-A unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms.
Sloan EA, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Raffel C, Berger MS, Chang SM, Reddy A, Ramani B, Ferris SP, Lee JC, Hofmann JW, Cho SJ, Horvai AE, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, Solomon DA
Brain Pathol 2021 Jul;31(4):e12918. Epub 2021 Jan 28 doi: 10.1111/bpa.12918. PMID: 33141488Free PMC Article
EWSR1 translocation in primary hyalinising clear cell carcinoma of the thymus.
Porubsky S, Rudolph B, Rückert JC, Küffer S, Ströbel P, Roden AC, Jain D, Tousseyn T, Van Veer H, Huang J, Antonicelli A, Kuo TT, Rosai J, Marx A; International Thymic Malignancy Interest Group (ITMIG)
Histopathology 2019 Sep;75(3):431-436. Epub 2019 Jul 19 doi: 10.1111/his.13890. PMID: 31050844

Diagnosis

Re-evaluating a historic cohort of sinonasal and skull base mucoepidermoid carcinoma: an institutional experience.
Hu C, Lin L, Ye M, Liu Y, Huang Q, Yuan C, Sun J, Sun H
Diagn Pathol 2024 Mar 1;19(1):46. doi: 10.1186/s13000-024-01466-5. PMID: 38429827Free PMC Article
Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
Maxillary clear cell odontogenic carcinoma with EWSR1-ATF1 fusion gene mimicking sclerosing odontogenic carcinoma: A case report and literature review.
Xie R, Wang W, Thomas AM, Li S, Qin H
Pathol Res Pract 2023 Jan;241:154257. Epub 2022 Nov 30 doi: 10.1016/j.prp.2022.154257. PMID: 36470043
Intracranial mesenchymal tumor with FET-CREB fusion-A unifying diagnosis for the spectrum of intracranial myxoid mesenchymal tumors and angiomatoid fibrous histiocytoma-like neoplasms.
Sloan EA, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Raffel C, Berger MS, Chang SM, Reddy A, Ramani B, Ferris SP, Lee JC, Hofmann JW, Cho SJ, Horvai AE, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, Solomon DA
Brain Pathol 2021 Jul;31(4):e12918. Epub 2021 Jan 28 doi: 10.1111/bpa.12918. PMID: 33141488Free PMC Article
Molecular Profiling of Clear Cell Myoepithelial Carcinoma of Salivary Glands With EWSR1 Rearrangement Identifies Frequent PLAG1 Gene Fusions But No EWSR1 Fusion Transcripts.
Skálová A, Agaimy A, Vanecek T, Baněčková M, Laco J, Ptáková N, Šteiner P, Majewska H, Biernat W, Corcione L, Eis V, Koshyk O, Vondrák J Jr, Michal M, Leivo I
Am J Surg Pathol 2021 Jan;45(1):1-13. doi: 10.1097/PAS.0000000000001591. PMID: 33027073

Therapy

Molecular Profiling of Clear Cell Myoepithelial Carcinoma of Salivary Glands With EWSR1 Rearrangement Identifies Frequent PLAG1 Gene Fusions But No EWSR1 Fusion Transcripts.
Skálová A, Agaimy A, Vanecek T, Baněčková M, Laco J, Ptáková N, Šteiner P, Majewska H, Biernat W, Corcione L, Eis V, Koshyk O, Vondrák J Jr, Michal M, Leivo I
Am J Surg Pathol 2021 Jan;45(1):1-13. doi: 10.1097/PAS.0000000000001591. PMID: 33027073

Prognosis

Re-evaluating a historic cohort of sinonasal and skull base mucoepidermoid carcinoma: an institutional experience.
Hu C, Lin L, Ye M, Liu Y, Huang Q, Yuan C, Sun J, Sun H
Diagn Pathol 2024 Mar 1;19(1):46. doi: 10.1186/s13000-024-01466-5. PMID: 38429827Free PMC Article
Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
Protein arginine methyltransferase 5 is essential for oncogene product EWSR1-ATF1-mediated gene transcription in clear cell sarcoma.
Li BX, David LL, Davis LE, Xiao X
J Biol Chem 2022 Oct;298(10):102434. Epub 2022 Aug 27 doi: 10.1016/j.jbc.2022.102434. PMID: 36041632Free PMC Article
Intracranial mesenchymal tumors with FET-CREB fusion are composed of at least two epigenetic subgroups distinct from meningioma and extracranial sarcomas.
Sloan EA, Gupta R, Koelsche C, Chiang J, Villanueva-Meyer JE, Alexandrescu S, Eschbacher JM, Wang W, Mafra M, Ud Din N, Carr-Boyd E, Watson M, Punsoni M, Oviedo A, Gilani A, Kleinschmidt-DeMasters BK, Coss DJ, Lopes MB, Reddy A, Mueller S, Cho SJ, Horvai AE, Lee JC, Pekmezci M, Tihan T, Bollen AW, Rodriguez FJ, Ellison DW, Perry A, von Deimling A, Chang SM, Berger MS, Solomon DA
Brain Pathol 2022 Jul;32(4):e13037. Epub 2021 Nov 25 doi: 10.1111/bpa.13037. PMID: 34821426Free PMC Article
Integrative sequencing discovers an ATF1-motif enriched molecular signature that differentiates hyalinizing clear cell carcinoma from mucoepidemoid carcinoma.
Heft Neal ME, Gensterblum-Miller E, Bhangale AD, Kulkarni A, Zhai J, Smith J, Brummel C, Foltin SK, Thomas D, Jiang H, McHugh JB, Brenner JC
Oral Oncol 2021 Jun;117:105270. Epub 2021 Apr 4 doi: 10.1016/j.oraloncology.2021.105270. PMID: 33827033Free PMC Article

Clinical prediction guides

Extraenteric Malignant Gastrointestinal Neuroectodermal Tumor-A Clinicopathologic and Molecular Genetic Study of 11 Cases.
Ulici V, Hornick JL, Davis JL, Mehrotra S, Meis JM, Halling KC, Fletcher CDM, Kao E, Folpe AL
Mod Pathol 2023 Jul;36(7):100160. Epub 2023 Mar 17 doi: 10.1016/j.modpat.2023.100160. PMID: 36934861
EWSR1-ATF1 dependent 3D connectivity regulates oncogenic and differentiation programs in Clear Cell Sarcoma.
Möller E, Praz V, Rajendran S, Dong R, Cauderay A, Xing YH, Lee L, Fusco C, Broye LC, Cironi L, Iyer S, Rengarajan S, Awad ME, Naigles B, Letovanec I, Ormas N, Finzi G, La Rosa S, Sessa F, Chebib I, Petur Nielsen G, Digklia A, Spentzos D, Cote GM, Choy E, Aryee M, Stamenkovic I, Boulay G, Rivera MN, Riggi N
Nat Commun 2022 Apr 27;13(1):2267. doi: 10.1038/s41467-022-29910-4. PMID: 35477713Free PMC Article
Cytokeratin-positive Malignant Tumor in the Abdomen With EWSR1/FUS-CREB Fusion: A Clinicopathologic Study of 8 Cases.
Shibayama T, Shimoi T, Mori T, Noguchi E, Honma Y, Hijioka S, Yoshida M, Ogawa C, Yonemori K, Yatabe Y, Yoshida A
Am J Surg Pathol 2022 Jan 1;46(1):134-146. doi: 10.1097/PAS.0000000000001742. PMID: 34049318
The clear cell sarcoma functional genomic landscape.
Panza E, Ozenberger BB, Straessler KM, Barrott JJ, Li L, Wang Y, Xie M, Boulet A, Titen SW, Mason CC, Lazar AJ, Ding L, Capecchi MR, Jones KB
J Clin Invest 2021 Aug 2;131(15) doi: 10.1172/JCI146301. PMID: 34156976Free PMC Article
Establishment and characterization of a clear cell odontogenic carcinoma cell line with EWSR1-ATF1 fusion gene.
Kujiraoka S, Tsunematsu T, Sato Y, Yoshida M, Ishikawa A, Tohyama R, Tanaka M, Kobayashi Y, Kondo T, Ushio A, Otsuka K, Kurosawa M, Saito M, Yamada A, Arakaki R, Nagai H, Nikai H, Takeuchi K, Nagao T, Miyamoto Y, Ishimaru N, Kudo Y
Oral Oncol 2017 Jun;69:46-55. Epub 2017 Apr 10 doi: 10.1016/j.oraloncology.2017.04.003. PMID: 28559020

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