|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We point out the telltale immunophenotypes: angiolymphoid hyperplasia with eosinophilia and EH (FOS-B/others negative), PM-HAE (FOS-B/AE1/AE3/others negative), epithelioid hemangioendothelioma (CAMTA-1 or TFE-3/others negative).
|
29266025 |
2019 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
A WWTR1-CAMTA1 fusion is present in most classic epithelioid hemangioendothelioma, regardless of their clinical behavior, suggesting that additional genetic abnormalities might be responsible in driving a more aggressive biology.
|
31537895 |
2019 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Care should be taken to identify such cells in limited biopsies; immunohistochemistry for CAMTA1, a specific and sensitive marker for EHE, can be confirmatory.
|
30664032 |
2019 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Characterization of the genetics of EHE is important because targeted therapies toward products of the specific WWTR1-CAMTA1 gene fusion may have an impact in the near future.
|
28855107 |
2018 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The 10% of EHEs that lack the TAZ⁻CAMTA1 fusion instead have a fusion of Yes-associated Protein (YAP) and Transcription Factor E3 (TFE3) genes (YAP-TFE3).
|
29996478 |
2018 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Of note, the FOSB-positive epithelioid hemangioendothelioma was negative for CAMTA1 and TFE3.
|
28009608 |
2017 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
The WWTR1 (protein is known as TAZ)-CAMTA1 (WC) fusion gene defines epithelioid hemangioendothelioma, a malignant vascular cancer.
|
25961935 |
2016 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Genetic characterization of several soft tissue tumour types that occur in the skin has resulted in the identification of diagnostically useful markers: ALK gene rearrangement with corresponding ALK protein expression by immunohistochemistry in epithelioid fibrous histiocytoma; the WWTR1-CAMTA1 fusion gene with CAMTA1 protein expression in epithelioid haemangioendothelioma; MYC amplification and overexpression in radiation-associated angiosarcoma; and EWSR1 gene rearrangement in cutaneous myoepithelial tumours.
|
26763770 |
2016 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Fluorescence in situ hybridization analysis showed CAMTA1-WWTR1 fusions in 4/7 low-grade and 23/23 intermediate-grade EHE (P<0.001).
|
25353289 |
2015 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Instead, WWTR1-CAMTA1 and YAP1-TFE3 fusion genes are found in almost all cases of epithelioid haemangioendothelioma.
|
26050962 |
2015 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Fourteen of 18 informative cases were positive for WWTR1-CAMTA1 fusion transcripts, four of which showed higher-grade cytological features termed by some as 'malignant EHE'.
|
25817592 |
2015 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Epithelioid hemangioendothelioma with extensive cystic change and CAMTA1 rearrangement.
|
24134632 |
2013 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Thus, we undertook a molecular analysis of six samples from two patients with multicentric hepatic EHE to test our hypothesis that the presence of identical breakpoints in WWTR1 and CAMTA1 support the monoclonal nature of multifocal EHE.
|
22429593 |
2012 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
The chimeric WWTR1/CAMTA1 transcription factor may represent a therapeutic target for EHE and offers the opportunity to shed light on the functions of two poorly characterized proteins.
|
21885404 |
2011 |
|
Epithelioid hemangioendothelioma, malignant
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our results demonstrate the presence of a WWTR1-CAMTA1 fusion in all EHE tested from bone, soft tissue, and visceral location (liver, lung) in keeping with a unique and specific pathological entity.
|
21584898 |
2011 |