MODERN METHODS OF DIAGNOSIS AND TREATMENT OF ATYPICAL TERATOID RHABDOID TUMORS CNS IN CHILDREN

Abstract

Atypical teratoid rhabdoid tumors (ATRT) of the CNS in children are a group of aggressive malignant neoplasms with extremely poor prognosis, and therefore these tumors are classified as IV (highest) grade of malignancy. At the time of diagnosis, 20% of patients are found to have metastasized to the brain and spinal cord. The pathogenesis of ATRT is based on the biallelic mutation of the SMARCB1 gene, more rarely SMARCA4 genes. Despite the use of intensive multimodal therapy, the survival rate of patients with ATRT remains low. Recently, several groups of scientists, based on epigenetic and transcriptional studies, have identified 3 molecular groups of ATRT with different therapeutic sensitivity to chemoradiation therapy and, accordingly, have a different prognosis of the disease. This article presents the current literature data on epidemiology, pathogenesis, clinic, diagnosis and therapy of patients with ATRT, and describes the directions of treatment.