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Atypical teratoid/rhabdoid tumor arising in pleomorphic xanthoastrocytoma

DC Field Value Language
dc.contributor.author홍순원-
dc.date.accessioned2015-01-06T17:27:51Z-
dc.date.available2015-01-06T17:27:51Z-
dc.date.issued2014-
dc.identifier.issn0919-6544-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/100027-
dc.description.abstractAtypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly malignant, true rhabdoid tumor in the central nervous system predominantly presenting in young children. AT/RT typically shows rhabdoid cells which can also be seen in other tumors, but it is differentiated from other tumors by the specific genetic alteration involving the SMARCB1 gene. Only a few cases of AT/RT arising in low-grade glioma have been reported. A 13-year-old girl presented with headache, dizziness, nausea and vomiting. A 4.7 cm cerebellar mass was found on MRI. The mass was totally removed. Histologically, the tumor revealed two distinct morphologic appearances: central areas of AT/RT containing rhabdoid cells and sarcomatous component in the background of pleomorphic xanthoastrocytoma (PXA). Immunohistochemically, PXA areas retained nuclear expression of INI-1 and low Ki-67 proliferation index, whereas AT/RT component showed loss of INI-1 nuclear expression and markedly elevated Ki-67 proliferation index. Epithelial membrane antigen (EMA), smooth muscle actin (SMA), and p53 protein were positive only in AT/RT. BRAF V600E mutation was identified in PXA by real-time polymerase chain reaction. We report a rare case of AT/RT arising in PXA which is supposed to progress by inactivation of INI-1 in a pre-existing PXA.-
dc.description.statementOfResponsibilityopen-
dc.format.extent398~405-
dc.relation.isPartOfNEUROPATHOLOGY-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.subject.MESHAdolescent-
dc.subject.MESHAstrocytoma/ultrastructure*-
dc.subject.MESHCerebellar Neoplasms/ultrastructure*-
dc.subject.MESHFemale-
dc.subject.MESHHumans-
dc.subject.MESHRhabdoid Tumor/ultrastructure*-
dc.subject.MESHTeratoma/ultrastructure*-
dc.titleAtypical teratoid/rhabdoid tumor arising in pleomorphic xanthoastrocytoma-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Pathology (병리학)-
dc.contributor.googleauthorJi Yun Jeong-
dc.contributor.googleauthorYeon-Lim Suh-
dc.contributor.googleauthorSoonWon Hong-
dc.identifier.doi10.1111/neup.12106-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA04411-
dc.relation.journalcodeJ02348-
dc.identifier.eissn1440-1789-
dc.identifier.pmid25268025-
dc.identifier.urlhttp://onlinelibrary.wiley.com/doi/10.1111/neup.12106/abstract-
dc.contributor.alternativeNameHong, Soon Won-
dc.contributor.affiliatedAuthorHong, Soon Won-
dc.rights.accessRightsfree-
dc.citation.volume34-
dc.citation.number4-
dc.citation.startPage398-
dc.citation.endPage405-
dc.identifier.bibliographicCitationNEUROPATHOLOGY, Vol.34(4) : 398-405, 2014-
dc.identifier.rimsid55093-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers

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