https://scholars.lib.ntu.edu.tw/handle/123456789/393248
DC Field | Value | Language |
---|---|---|
dc.contributor.author | YUNG-MING JENG | en |
dc.contributor.author | JAU-YU LIAU | en |
dc.contributor.author | JEN-CHIEH LEE | en |
dc.contributor.author | CHING-YAO YANG | en |
dc.creator | Liau, J.-Y. and Tsai, J.-H. and Yang, C.-Y. and Lee, J.-C. and Liang, C.-W. and Hsu, H.-H. and Jeng, Y.-M. | - |
dc.date.accessioned | 2018-09-10T15:23:25Z | - |
dc.date.available | 2018-09-10T15:23:25Z | - |
dc.date.issued | 2015 | - |
dc.identifier.uri | http://www.scopus.com/inward/record.url?eid=2-s2.0-84940460985&partnerID=MN8TOARS | - |
dc.identifier.uri | http://scholars.lib.ntu.edu.tw/handle/123456789/393248 | - |
dc.description.abstract | Summary Alternative lengthening of telomeres (ALT) is a mechanism using homologous recombination to maintain telomere length and sustain limitless replicability of cancer cells. Recently, ALT has been found to be associated with inactivation of either α-thalassemia/mental retardation syndrome X-linked (ATRX) or death domain-associated (DAXX) protein. In this study, 119 tumors (88 angiosarcomas, 11 epithelioid hemangioendotheliomas, and 20 Kaposi sarcomas) were analyzed to determine the ALT status, its relationship to loss of ATRX/DAXX expression, and the clinicopathological features. In addition, the mutation status in the telomerase reverse transcriptase gene (TERT) promoter was also studied. Loss of ATRX expression was observed in 21% (16/77) of the primary angiosarcomas and 9% (1/11) of epithelioid hemangioendotheliomas. DAXX expression was intact in all but 2 ATRX-deficient angiosarcomas. Telomere-specific fluorescence in situ hybridization assay showed 28% (17/61) of the primary angiosarcomas were ALT positive. Remarkably, ALT was highly associated with loss of ATRX expression: all but 2 ALT-positive angiosarcomas were ATRX deficient. Notably, hepatic angiosarcomas were frequently ATRX deficient (8/13) and/or ALT positive (8/12). None of the secondary angiosarcomas were ATRX/DAXX deficient or ALT positive. The only ATRX-deficient epithelioid hemangioendothelioma was positive for ALT. Forty-seven angiosarcomas were tested for TERT promoter mutation. Despite the fact that angiosarcoma occurs most commonly in sun-damaged skin, mutation was detected in only 1 radiation-associated angiosarcoma (2%). We conclude that ALT is an important telomere maintenance mechanism in primary angiosarcomas. This feature is highly associated with loss of ATRX expression and is frequently observed in hepatic angiosarcomas. ? 2015 Elsevier Inc. | - |
dc.language | en | en |
dc.relation.ispartof | Human Pathology | - |
dc.source | AH-Scopus to ORCID | - |
dc.subject.other | alpha thalassemia mental retardation syndrome X linked protein; death domain associated protein; protein; telomerase reverse transcriptase; unclassified drug; ATRX protein, human; DNA helicase; nuclear protein; telomerase; TERT protein, human; tumor marker; adult; aged; Article; breast cancer; clinical feature; cystosarcoma phylloides; female; fluorescence in situ hybridization; follow up; gene mutation; hemangioendothelioma; hepatic angiosarcoma; histopathology; human; human tissue; Kaposi sarcoma; liver sarcoma; major clinical study; male; malignant peripheral nerve sheath tumor; mitosis rate; nasopharynx carcinoma; phenotype; promoter region; protein expression; survival; telomere homeostasis; undifferentiated carcinoma; uterine cervix carcinoma; vascular tumor; angiosarcoma; down regulation; enzymology; genetics; immunohistochemistry; liver tumor; middle aged; mortality; mutation; nucleotide sequence; pathology; prognosis; skin tumor; telomere; Adult; Aged; DNA Helicases; DNA Mutational Analysis; Down-Regulation; Female; Hemangioendothelioma, Epithelioid; Hemangiosarcoma; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Liver Neoplasms; Male; Middle Aged; Mutation; Nuclear Proteins; Prognosis; Promoter Regions, Genetic; Sarcoma, Kaposi; Skin Neoplasms; Telomerase; Telomere; Telomere Homeostasis; Tumor Markers, Biological | - |
dc.subject.other | [SDGs]SDG3 | - |
dc.title | Alternative lengthening of telomeres phenotype in malignant vascular tumors is highly associated with loss of ATRX expression and is frequently observed in hepatic angiosarcomas | - |
dc.type | journal article | en |
dc.identifier.doi | 10.1016/j.humpath.2015.05.019 | - |
dc.relation.pages | 1360-1366 | - |
dc.relation.journalvolume | 46 | - |
dc.relation.journalissue | 9 | - |
item.openairecristype | http://purl.org/coar/resource_type/c_6501 | - |
item.openairetype | journal article | - |
item.cerifentitytype | Publications | - |
item.grantfulltext | none | - |
item.fulltext | no fulltext | - |
Appears in Collections: | 醫學系 |
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