The research progress on atypical teratoid/rhabdoid tumor of the central nervous system in children / 中国神经精神疾病杂志

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.