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Epithelioid Hemangioendothelioma with YAP1-TFE3 Gene Fusion

MedGen UID:
1788768
Concept ID:
C5447847
Neoplastic Process
Synonym: Epithelioid Hemangioendothelioma with YAP1::TFE3 Gene Fusion

Definition

Epithelioid hemangioendothelioma characterized by a YAP1-TFE3 gene fusion. This gene fusion occurs in a subset of epithelioid hemangioendotheliomas characterized by the presence of well-formed vessels lined by epithelioid endothelial cells with abundant eosinophilic cytoplasm. Patients with YAP1-TFE3 gene fusion tumors tend to be younger than those with a WWTR1-CAMTA1 gene fusion. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Hemangioendothelioma with YAP1-TFE3 Gene Fusion

Recent clinical studies

Etiology

Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Lee SJ, Yang WI, Chung WS, Kim SK
Oncotarget 2016 Feb 16;7(7):7480-8. doi: 10.18632/oncotarget.7060. PMID: 26840265Free PMC Article
Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, Chen HW, Pathan N, Krausz T, Dickson BC, Weinreb I, Rubin MA, Hameed M, Fletcher CD
Genes Chromosomes Cancer 2013 Aug;52(8):775-84. Epub 2013 Jun 5 doi: 10.1002/gcc.22073. PMID: 23737213Free PMC Article

Diagnosis

Vazzano JL, Patton A, Tinoco G, Iwenofu OH
Int J Surg Pathol 2022 Aug;30(5):557-563. Epub 2022 Jan 31 doi: 10.1177/10668969211070174. PMID: 35098778
Bourgeau M, Martinez A, Deeb KK, Reid MD, Lewis M, Point du Jour KS, Lai J, Shi Q
Diagn Cytopathol 2021 Dec;49(12):E447-E452. Epub 2021 Aug 19 doi: 10.1002/dc.24853. PMID: 34411462
Anderson WJ, Fletcher CDM, Hornick JL
Mod Pathol 2021 Nov;34(11):2036-2042. Epub 2021 Jun 19 doi: 10.1038/s41379-021-00854-2. PMID: 34148063
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851
Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, Chen HW, Pathan N, Krausz T, Dickson BC, Weinreb I, Rubin MA, Hameed M, Fletcher CD
Genes Chromosomes Cancer 2013 Aug;52(8):775-84. Epub 2013 Jun 5 doi: 10.1002/gcc.22073. PMID: 23737213Free PMC Article

Prognosis

Patton A, Bridge JA, Liebner D, Chung C, Iwenofu OH
Genes Chromosomes Cancer 2022 Apr;61(4):194-199. Epub 2021 Dec 14 doi: 10.1002/gcc.23018. PMID: 34874592
Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Lee SJ, Yang WI, Chung WS, Kim SK
Oncotarget 2016 Feb 16;7(7):7480-8. doi: 10.18632/oncotarget.7060. PMID: 26840265Free PMC Article
Doyle LA, Fletcher CD, Hornick JL
Am J Surg Pathol 2016 Jan;40(1):94-102. doi: 10.1097/PAS.0000000000000511. PMID: 26414223
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851

Clinical prediction guides

Rosenbaum E, Jadeja B, Xu B, Zhang L, Agaram NP, Travis W, Singer S, Tap WD, Antonescu CR
Mod Pathol 2020 Apr;33(4):591-602. Epub 2019 Sep 19 doi: 10.1038/s41379-019-0368-8. PMID: 31537895Free PMC Article
Lee SJ, Yang WI, Chung WS, Kim SK
Oncotarget 2016 Feb 16;7(7):7480-8. doi: 10.18632/oncotarget.7060. PMID: 26840265Free PMC Article
Doyle LA, Fletcher CD, Hornick JL
Am J Surg Pathol 2016 Jan;40(1):94-102. doi: 10.1097/PAS.0000000000000511. PMID: 26414223
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851

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