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Atypical teratoid/rhabdoid tumor arising in pleomorphic xanthoastrocytoma

Authors
 Ji Yun Jeong  ;  Yeon-Lim Suh  ;  SoonWon Hong 
Citation
 NEUROPATHOLOGY, Vol.34(4) : 398-405, 2014 
Journal Title
 NEUROPATHOLOGY 
ISSN
 0919-6544 
Issue Date
2014
MeSH
Adolescent ; Astrocytoma/ultrastructure* ; Cerebellar Neoplasms/ultrastructure* ; Female ; Humans ; Rhabdoid Tumor/ultrastructure* ; Teratoma/ultrastructure*
Abstract
Atypical 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.
Full Text
http://onlinelibrary.wiley.com/doi/10.1111/neup.12106/abstract
DOI
10.1111/neup.12106
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
Hong, Soon Won(홍순원) ORCID logo https://orcid.org/0000-0002-0324-2414
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/100027
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