The research progress on atypical teratoid/rhabdoid tumor of the central nervous system in children
10.3969/j.issn.1002-0152.2023.11.008
- VernacularTitle:儿童中枢神经系统非典型畸胎样/横纹肌样瘤研究进展
- Author:
Wenlong GAO
1
;
Pan GOU
;
Ping LIANG
Author Information
1. 重庆医科大学附属儿童医院神经外科,国家儿童健康与疾病临床医学研究中心,儿童发育疾病研究教育部重点实验室,儿科学重庆市重点实验室(重庆 400014)
- Keywords:
Children;
Atypical teratoid rhabdoid tumor;
Advances in treatment;
Pathogenesis;
Diagnosis;
Treat-ment;
SMARCB1
- From:
Chinese Journal of Nervous and Mental Diseases
2023;49(11):682-688
- CountryChina
- Language:Chinese
-
Abstract:
Atypical teratoid/rhabdoid tumor(AT/RT)is a rare and highly malignant embryonal tumor of the central nervous system in children,characterized by diverse histological morphology,high malignancy,rapid clinical progression,and poor prognosis for affected children.The pathogenesis of AT/RT involves mutations in chromosomes and genes,particularly the loss of function of the SMARCB1 gene.The diagnosis of AT/RT primarily relies on histological and immunohistochemical analysis.Currently,there is no standardized treatment protocol for AT/RT.The main treatment modalities include surgery,chemotherapy,radiotherapy,as well as emerging targeted therapy and immunotherapy.Despite progress in research and clinical trials on AT/RT in recent years,the prognosis for affected children remains poor,necessitating further research to develop more effective treatment strategies to improve patient outcomes.
