Clinical and pathological features of atypical teratoid/rhabdoid tumor in the central nervous system and diagnostic significance of INI1 expression in the tumor:report of one case
10.3760/cma.j.issn.1671-8925.2010.03.019
- VernacularTitle:中枢神经系统非典型畸胎样/横纹肌样瘤临床病理特征及INI1的表达(附一例报道)
- Author:
Jun-Mei WANG
1
;
Lin LUO
;
Yu-Jin SU
;
Ke DAI
;
Jing-Yi FANG
;
Sheng-Lin YUE
;
Gui-Lin LI
Author Information
1. 首都医科大学
- Keywords:
Atypical teratoid/rhab doid tumor;
Central nervous system;
INI1
- From:
Chinese Journal of Neuromedicine
2010;9(3):291-294,298
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical and pathological features of pediatric CNS atypical teratoid/rhabdoid tumor(AT/RT)and evaluate the role of INI1 antibody on the differential diagnosis and prognosis of AT/RT.Methods The clinical data and pathological features of 12-year-old boy with AT/RT were concluded.The protein expression of INI1 was detected by immunohistochemistry;follow-up was performed and relative documents of that disease were compared;the above data were retrospectively analyzed.Results Mioroanatomy showed typical teratoid cells and primitive mesenchymal cells in the tumor.Both of these 2 tumor components displayed positive reactions of vimentin and CD99.The SMA and EMA positive expressions were noted in the teratoid cells.Immunohistochemical staining demonstrated that INI1 antibody was nuclear negative in tumor cells,but positive within endothelial cells of the blood.Follow-up information showed the patient was dead 2 months after the operation.Conclusions CNS atypical AT/RT is highly aggressive tumor.The appearance of rhabdoid cells is its important morphologic features and its main differential diagnosis evidence.Mesenchymal components and the various results of immunohistochemical staining indicate that AT/RT tumor cells,having the character of the embryonal tumors,have various differentiation potential.The expression of INI1 antibody suggests that a mutation or deletion in the INI1 gene occurs in the tumor,which has important value in diagnosing and differentiating the tumor.
