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WWTR1-CAMTA1 Fusion Protein Expression

MedGen UID:
888718
•Concept ID:
C4053855
•
Cell or Molecular Dysfunction

Definition

A fusion protein that results from a t(1;3)(p36;q23-25). This fusion protein expression is seen in virtually all cases of epithelioid hemangioendothelioma and is unique to this neoplasm. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWWTR1-CAMTA1 Fusion Protein Expression

Professional guidelines

PubMed

CAMTA-1 Expression in 24 Cases of Hepatic Epithelioid Hemangioendothelioma in a Single Institute: Diagnostic Utility for Differential Diagnosis from Hepatic Angiosarcoma.
Jung H, Kim HN, Jang Y, Park CK, Ha SY
In Vivo 2019 Nov-Dec;33(6):2293-2297. doi: 10.21873/invivo.11736. PMID: 31662570Free PMC Article

Recent clinical studies

Etiology

Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements.
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
Thoracic epithelioid malignant vascular tumors: a clinicopathologic study of 52 cases with emphasis on pathologic grading and molecular studies of WWTR1-CAMTA1 fusions.
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
Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma.
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

Common Secondary Genomic Variants Associated With Advanced Epithelioid Hemangioendothelioma.
Seligson ND, Awasthi A, Millis SZ, Turpin BK, Meyer CF, Grand'Maison A, Liebner DA, Hays JL, Chen JL
JAMA Netw Open 2019 Oct 2;2(10):e1912416. doi: 10.1001/jamanetworkopen.2019.12416. PMID: 31577358Free PMC Article
Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms.
Costigan DC, Doyle LA
Histopathology 2016 May;68(6):776-95. Epub 2016 Mar 1 doi: 10.1111/his.12930. PMID: 26763770
Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics.
Doyle LA, Fletcher CD, Hornick JL
Am J Surg Pathol 2016 Jan;40(1):94-102. doi: 10.1097/PAS.0000000000000511. PMID: 26414223
CAMTA1 is a useful immunohistochemical marker for diagnosing epithelioid haemangioendothelioma.
Shibuya R, Matsuyama A, Shiba E, Harada H, Yabuki K, Hisaoka M
Histopathology 2015 Dec;67(6):827-35. Epub 2015 May 22 doi: 10.1111/his.12713. PMID: 25879300
CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers.
Yusıflı Z, Kösemehmetoğlu K
Turk Patoloji Derg 2014;30(3):159-65. doi: 10.5146/tjpath.2014.01272. PMID: 25110239

Prognosis

Common Secondary Genomic Variants Associated With Advanced Epithelioid Hemangioendothelioma.
Seligson ND, Awasthi A, Millis SZ, Turpin BK, Meyer CF, Grand'Maison A, Liebner DA, Hays JL, Chen JL
JAMA Netw Open 2019 Oct 2;2(10):e1912416. doi: 10.1001/jamanetworkopen.2019.12416. PMID: 31577358Free PMC Article
Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements.
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
The 2015 World Health Organization Classification of Tumors of the Pleura: Advances since the 2004 Classification.
Galateau-Salle F, Churg A, Roggli V, Travis WD; World Health Organization Committee for Tumors of the Pleura
J Thorac Oncol 2016 Feb;11(2):142-54. doi: 10.1016/j.jtho.2015.11.005. PMID: 26811225
Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms.
Costigan DC, Doyle LA
Histopathology 2016 May;68(6):776-95. Epub 2016 Mar 1 doi: 10.1111/his.12930. PMID: 26763770
Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics.
Doyle LA, Fletcher CD, Hornick JL
Am J Surg Pathol 2016 Jan;40(1):94-102. doi: 10.1097/PAS.0000000000000511. PMID: 26414223

Clinical prediction guides

Common Secondary Genomic Variants Associated With Advanced Epithelioid Hemangioendothelioma.
Seligson ND, Awasthi A, Millis SZ, Turpin BK, Meyer CF, Grand'Maison A, Liebner DA, Hays JL, Chen JL
JAMA Netw Open 2019 Oct 2;2(10):e1912416. doi: 10.1001/jamanetworkopen.2019.12416. PMID: 31577358Free PMC Article
Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements.
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
The 2015 World Health Organization Classification of Tumors of the Pleura: Advances since the 2004 Classification.
Galateau-Salle F, Churg A, Roggli V, Travis WD; World Health Organization Committee for Tumors of the Pleura
J Thorac Oncol 2016 Feb;11(2):142-54. doi: 10.1016/j.jtho.2015.11.005. PMID: 26811225
Nuclear Expression of CAMTA1 Distinguishes Epithelioid Hemangioendothelioma From Histologic Mimics.
Doyle LA, Fletcher CD, Hornick JL
Am J Surg Pathol 2016 Jan;40(1):94-102. doi: 10.1097/PAS.0000000000000511. PMID: 26414223
CAMTA1 immunostaining is not useful in differentiating epithelioid hemangioendothelioma from its potential mimickers.
Yusıflı Z, Kösemehmetoğlu K
Turk Patoloji Derg 2014;30(3):159-65. doi: 10.5146/tjpath.2014.01272. PMID: 25110239

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