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FUS Gene Rearrangement

MedGen UID:
1775825
•Concept ID:
C5420787
•
Cell or Molecular Dysfunction
Synonyms: FUS Rearrangement; FUS RNA Binding Protein Gene Rearrangement; FUS1 Gene Rearrangement; Fused In Sarcoma Gene Rearrangement; Fusion Gene In Myxoid Liposarcoma Gene Rearrangement; TLS Gene Rearrangement; Translocated In Liposarcoma Gene Rearrangement"

Definition

A molecular abnormality indicating rearrangement of the FUS gene. [from NCI]

Professional guidelines

PubMed

In-depth Genetic Analysis of Sclerosing Epithelioid Fibrosarcoma Reveals Recurrent Genomic Alterations and Potential Treatment Targets.
Arbajian E, Puls F, Antonescu CR, Amary F, Sciot R, Debiec-Rychter M, Sumathi VP, Järås M, Magnusson L, Nilsson J, Hofvander J, Mertens F
Clin Cancer Res 2017 Dec 1;23(23):7426-7434. Epub 2017 Sep 22 doi: 10.1158/1078-0432.CCR-17-1856. PMID: 28939748

Recent clinical studies

Etiology

Epithelioid mesenchymal neoplasm with FUS::CREM gene fusion in the tongue: Report of a rare and challenging diagnosis.
Sun Y, Liu D, Chen X, Zhang J, Yang S
Oral Surg Oral Med Oral Pathol Oral Radiol 2023 May;135(5):e108-e113. Epub 2022 Dec 19 doi: 10.1016/j.oooo.2022.12.003. PMID: 36697296
Expanding the Spectrum of Intraosseous Rhabdomyosarcoma: Correlation Between 2 Distinct Gene Fusions and Phenotype.
Agaram NP, Zhang L, Sung YS, Cavalcanti MS, Torrence D, Wexler L, Francis G, Sommerville S, Swanson D, Dickson BC, Suurmeijer AJH, Williamson R, Antonescu CR
Am J Surg Pathol 2019 May;43(5):695-702. doi: 10.1097/PAS.0000000000001227. PMID: 30720533Free PMC Article
Cutaneous syncytial myoepithelioma: clinicopathologic characterization in a series of 38 cases.
Jo VY, Antonescu CR, Zhang L, Dal Cin P, Hornick JL, Fletcher CD
Am J Surg Pathol 2013 May;37(5):710-8. doi: 10.1097/PAS.0b013e3182772bba. PMID: 23588365Free PMC Article
MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement.
Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, Hornick JL
Am J Surg Pathol 2012 Oct;36(10):1444-51. doi: 10.1097/PAS.0b013e3182562bf8. PMID: 22982887
Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation.
Matsumura T, Yamaguchi T, Tochigi N, Wada T, Yamashita T, Hasegawa T
J Clin Pathol 2010 Feb;63(2):124-8. doi: 10.1136/jcp.2009.072256. PMID: 20154033

Diagnosis

Epithelioid mesenchymal neoplasm with FUS::CREM gene fusion in the tongue: Report of a rare and challenging diagnosis.
Sun Y, Liu D, Chen X, Zhang J, Yang S
Oral Surg Oral Med Oral Pathol Oral Radiol 2023 May;135(5):e108-e113. Epub 2022 Dec 19 doi: 10.1016/j.oooo.2022.12.003. PMID: 36697296
Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature.
Vallejo-Benítez A, Rodríguez-Zarco E, Carrasco SP, Pereira-Gallardo S, Brugal Molina J, García-Escudero A, Robles Frías A, Marcilla D, González-Cámpora R
Ann Diagn Pathol 2017 Oct;30:8-11. Epub 2017 May 11 doi: 10.1016/j.anndiagpath.2017.05.002. PMID: 28965630
Paravertebral Low-grade Fibromyxoid Sarcoma with Supernumerary Ring Chromosome: Case Report and Literature Review.
Chien YC, Károlyi K, Kovács I
Ann Clin Lab Sci 2016 Winter;46(1):90-6. PMID: 26927350
MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement.
Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, Hornick JL
Am J Surg Pathol 2012 Oct;36(10):1444-51. doi: 10.1097/PAS.0b013e3182562bf8. PMID: 22982887
MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma.
Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL
Am J Surg Pathol 2011 May;35(5):733-41. doi: 10.1097/PAS.0b013e318210c268. PMID: 21415703

Prognosis

Primary low-grade fibromyxoid sarcoma of the breast: a rare case report with immunohistochemical and fluorescence in situ hybridization detection.
Zhang Y, Wan D, Gao F
Hum Pathol 2018 Sep;79:208-211. Epub 2018 Feb 25 doi: 10.1016/j.humpath.2018.02.013. PMID: 29486291
Low-grade fibromyxoid sarcoma of the external anal sphincter: a case report.
Ban LK, Tseng AH, Huang SH, Lee HH
World J Surg Oncol 2017 May 30;15(1):109. doi: 10.1186/s12957-017-1174-8. PMID: 28558780Free PMC Article
Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation.
Matsumura T, Yamaguchi T, Tochigi N, Wada T, Yamashita T, Hasegawa T
J Clin Pathol 2010 Feb;63(2):124-8. doi: 10.1136/jcp.2009.072256. PMID: 20154033

Clinical prediction guides

Primary low-grade fibromyxoid sarcoma of the breast: a rare case report with immunohistochemical and fluorescence in situ hybridization detection.
Zhang Y, Wan D, Gao F
Hum Pathol 2018 Sep;79:208-211. Epub 2018 Feb 25 doi: 10.1016/j.humpath.2018.02.013. PMID: 29486291
Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature.
Vallejo-Benítez A, Rodríguez-Zarco E, Carrasco SP, Pereira-Gallardo S, Brugal Molina J, García-Escudero A, Robles Frías A, Marcilla D, González-Cámpora R
Ann Diagn Pathol 2017 Oct;30:8-11. Epub 2017 May 11 doi: 10.1016/j.anndiagpath.2017.05.002. PMID: 28965630
MUC4 is a sensitive and extremely useful marker for sclerosing epithelioid fibrosarcoma: association with FUS gene rearrangement.
Doyle LA, Wang WL, Dal Cin P, Lopez-Terrada D, Mertens F, Lazar AJ, Fletcher CD, Hornick JL
Am J Surg Pathol 2012 Oct;36(10):1444-51. doi: 10.1097/PAS.0b013e3182562bf8. PMID: 22982887
Angiomatoid fibrous histiocytoma including cases with pleomorphic features analysed by fluorescence in situ hybridisation.
Matsumura T, Yamaguchi T, Tochigi N, Wada T, Yamashita T, Hasegawa T
J Clin Pathol 2010 Feb;63(2):124-8. doi: 10.1136/jcp.2009.072256. PMID: 20154033

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