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Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Gene Fusion

MedGen UID:
1783904
Concept ID:
C5447846
Neoplastic Process
Synonym: Epithelioid Hemangioendothelioma with WWTR1::CAMTA1 Gene Fusion

Definition

Epithelioid hemangioendothelioma characterized by a t(1;3)(p36;q23-q25) translocation, resulting in a WWTR1-CAMTA1 gene fusion. This translocation and associated gene fusion occur in more than 90% of epithelioid hemangioendotheliomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEpithelioid Hemangioendothelioma with WWTR1-CAMTA1 Gene Fusion

Professional guidelines

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
Anderson T, Zhang L, Hameed M, Rusch V, Travis WD, Antonescu CR
Am J Surg Pathol 2015 Jan;39(1):132-9. doi: 10.1097/PAS.0000000000000346. PMID: 25353289Free 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
Suurmeijer AJH, Dickson BC, Swanson D, Sung YS, Zhang L, Antonescu CR
Genes Chromosomes Cancer 2020 Jul;59(7):389-395. Epub 2020 Mar 20 doi: 10.1002/gcc.22839. PMID: 32170768Free 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
Yusıflı Z, Kösemehmetoğlu K
Turk Patoloji Derg 2014;30(3):159-65. doi: 10.5146/tjpath.2014.01272. PMID: 25110239
Antonescu C
Mod Pathol 2014 Jan;27 Suppl 1:S30-8. doi: 10.1038/modpathol.2013.176. PMID: 24384851

Therapy

Hahn E, Dickson BC, Gupta AA, Nofech-Mozes S
Genes Chromosomes Cancer 2021 Feb;60(2):112-115. Epub 2020 Nov 10 doi: 10.1002/gcc.22912. PMID: 33125182

Prognosis

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
Costigan DC, Doyle LA
Histopathology 2016 May;68(6):776-95. Epub 2016 Mar 1 doi: 10.1111/his.12930. PMID: 26763770
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

Seavey CN, Pobbati AV, Hallett A, Ma S, Reynolds JP, Kanai R, Lamar JM, Rubin BP
Genes Dev 2021 Apr 1;35(7-8):512-527. Epub 2021 Mar 25 doi: 10.1101/gad.348220.120. PMID: 33766982Free PMC Article
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

Supplemental Content

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