Atypical teratoid/rhabdoid tumors of the central nervous system in children: the state of the problem today. Literature review

Abstract

Atypical teratoid/rhabdoid tumors (AT/RT) are a group of rare highly aggressive malignant tumors in young patients. Among all the malignant tumors of the central nervous system (CNS) in children, they are 1–2 %, which, due to the small number of groups, makes it difficult to develop uniform recommendations for antitumor therapy. The molecular genetic profile of AT/RT, which largely determines the characteristics of the disease, has been studied sufficiently. Despite the large number of ongoing clinical studies, the results of treatment of AT/RT CNS in the world today remain unsatisfactory. The early age of patients limits the use of radiation therapy, which leads to the need to intensify chemotherapy regimens and to choose the optimal strategy in the toxicity – benefit ratio. The article describes modern approaches to the treatment of central nervous system disorders in children, presents the results of studies with the largest number of included patients, using the multimodal treatment strategy, identifies current trends in targeted therapy.Conflict of interest. The authors declare no conflict of interest.Funding. The study was performed without external funding.

Long-Term Survival of a Child with Atypical Teratoid-Rhabdoid Tumor and Acute Lymphoblastic Leukemia: A Case Report

“…The division of AT/RT into three molecular groups revived the interest of researchers in this seemingly unpromising problem [27]. ATRT-SHH represents the most common molecular group [28], and overexpression signaling pathway of the SHH signaling pathway and Notch transmission pathways (a type of transmembrane protein) are a characteristic feature of the above subgroup. The issue of predictive application of this classification remains debatable.…”

“…The issue of predictive application of this classification remains debatable. The results of clinical trials and registries are contradictory: in the EU-RHAB registry (European Registry of AT/RT), the survival rate in children with a verified molecular group of ATRT-SHH and ATRT-MYC was worse than in ATRT-TYR [28], while in the ACNS0333 study, a longer disease-free period and longer survival rate in children with the ATRT-SHH molecular subgroup were reported [29,30]. In 2022, the ATRT-SHH molecular group was divided into three subgroups: SHH-1a, SHH-1b, and SHH-2, which differ in the age of the patient's tumor and its localization [31].…”

Atypical teratoid-rhabdoid tumors: molecular genetics feutures, perspectives of treatment. Review of literature

“…Девять пациентов с М0-М3-стадиями получали лечение по данному протоколу. Пятилетняя ОВ в данной группе составила 100 %, БСВ -88,9 ± 10,5 % [24,48].…”