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TFCP2 Gene Rearrangement

MedGen UID:
1791330
•Concept ID:
C5554889
•
Cell or Molecular Dysfunction
Synonyms: LSF Gene Rearrangement; SEF Gene Rearrangement; Transcription Factor CP2 Gene Rearrangement

Definition

A molecular abnormality indicating rearrangement of the TFCP2 gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTFCP2 Gene Rearrangement

Recent clinical studies

Etiology

Intraosseous Spindle Cell/Epithelioid Rhabdomyosarcoma with TFCP2 Rearrangement: A Recent Recognized Subtype with Partial Response to Alectinib.
Valério E, Furtado Costa JL, Perez Fraile NM, Credidio CH, Taveira Garcia MR, Neto CS, Costa FD
Int J Surg Pathol 2023 Aug;31(5):861-865. Epub 2022 Dec 6 doi: 10.1177/10668969221140397. PMID: 36474403
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

Diagnosis

Mandibular rhabdomyosarcoma with TFCP2 rearrangement and osteogenic differentiation: a case misdiagnosed as fibrous dysplasia or low-grade central osteosarcoma.
Chen F, Wang J, Sun Y, Zhang J
Oral Surg Oral Med Oral Pathol Oral Radiol 2024 Jun;137(6):e143-e149. Epub 2024 Jan 11 doi: 10.1016/j.oooo.2023.12.792. PMID: 38637236
Rhabdomyosarcoma With FUS::TFCP2 Fusion in the Scalp: A Rare Case Report Depicting Round and Spindle cell Morphology.
Ishiyama T, Kato I, Ito J, Matsumura M, Saito K, Kawabata Y, Kato S, Takeyama M, Fujii S
Int J Surg Pathol 2023 Aug;31(5):805-812. Epub 2022 Nov 27 doi: 10.1177/10668969221137517. PMID: 36437645
Clinicopathological features of rhabdomyosarcoma with novel FET::TFCP2 and TIMP3::ALK fusion: report of two cases and literature review.
Duan FL, Yang H, Gong X, Zuo Z, Qin S, Ji J, Zhou C, Dai J, Guo P, Liu Y
Histopathology 2023 Feb;82(3):478-484. Epub 2022 Nov 21 doi: 10.1111/his.14833. PMID: 36350070
Epithelioid and Spindle Cell Rhabdomyosarcoma of the Oral Mucosa with FUS Rearrangement.
Ochsner AR, Foss RD
Head Neck Pathol 2022 Sep;16(3):823-827. Epub 2022 Mar 30 doi: 10.1007/s12105-022-01424-x. PMID: 35353344Free PMC Article
Clinicopathologic and Genomic Characterization of Inflammatory Myofibroblastic Tumors of the Head and Neck: Highlighting a Novel Fusion and Potential Diagnostic Pitfall.
Kerr DA, Thompson LDR, Tafe LJ, Jo VY, Neyaz A, Divakar P, Paydarfar JA, Pastel DA, Shirai K, John I, Seethala RR, Salgado CM, Deshpande V, Bridge JA, Kashofer K, Brčić I, Linos K
Am J Surg Pathol 2021 Dec 1;45(12):1707-1719. doi: 10.1097/PAS.0000000000001735. PMID: 34001695

Prognosis

Epithelioid and spindle rhabdomyosarcoma with TFCP2 rearrangement in abdominal wall: a distinctive entity with poor prognosis.
Li Y, Li D, Wang J, Tang J
Diagn Pathol 2023 Mar 30;18(1):41. doi: 10.1186/s13000-023-01330-y. PMID: 36998041Free PMC Article
Clinicopathological features of rhabdomyosarcoma with novel FET::TFCP2 and TIMP3::ALK fusion: report of two cases and literature review.
Duan FL, Yang H, Gong X, Zuo Z, Qin S, Ji J, Zhou C, Dai J, Guo P, Liu Y
Histopathology 2023 Feb;82(3):478-484. Epub 2022 Nov 21 doi: 10.1111/his.14833. PMID: 36350070
Clinicopathologic and Genomic Characterization of Inflammatory Myofibroblastic Tumors of the Head and Neck: Highlighting a Novel Fusion and Potential Diagnostic Pitfall.
Kerr DA, Thompson LDR, Tafe LJ, Jo VY, Neyaz A, Divakar P, Paydarfar JA, Pastel DA, Shirai K, John I, Seethala RR, Salgado CM, Deshpande V, Bridge JA, Kashofer K, Brčić I, Linos K
Am J Surg Pathol 2021 Dec 1;45(12):1707-1719. doi: 10.1097/PAS.0000000000001735. PMID: 34001695

Clinical prediction guides

Clinicopathological features of rhabdomyosarcoma with novel FET::TFCP2 and TIMP3::ALK fusion: report of two cases and literature review.
Duan FL, Yang H, Gong X, Zuo Z, Qin S, Ji J, Zhou C, Dai J, Guo P, Liu Y
Histopathology 2023 Feb;82(3):478-484. Epub 2022 Nov 21 doi: 10.1111/his.14833. PMID: 36350070
Clinicopathologic and Genomic Characterization of Inflammatory Myofibroblastic Tumors of the Head and Neck: Highlighting a Novel Fusion and Potential Diagnostic Pitfall.
Kerr DA, Thompson LDR, Tafe LJ, Jo VY, Neyaz A, Divakar P, Paydarfar JA, Pastel DA, Shirai K, John I, Seethala RR, Salgado CM, Deshpande V, Bridge JA, Kashofer K, Brčić I, Linos K
Am J Surg Pathol 2021 Dec 1;45(12):1707-1719. doi: 10.1097/PAS.0000000000001735. PMID: 34001695

Recent systematic reviews

Epithelioid and spindle rhabdomyosarcoma with TFCP2 rearrangement in abdominal wall: a distinctive entity with poor prognosis.
Li Y, Li D, Wang J, Tang J
Diagn Pathol 2023 Mar 30;18(1):41. doi: 10.1186/s13000-023-01330-y. PMID: 36998041Free PMC Article

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