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Abstract
Introduction. Atypical teratoid rhabdoid tumors (ATRO) are extremely rare aggressive tumors of childhood with predominant occurrence in patients under 3 years of age and primary localization within the central nervous system (CNS). Despite intensive multimodal treatment approaches, the prognosis in this cohort of patients remains unsatisfactory.
Case description. We present a case of an aggressive primary metastatic ATRT in combination with low-grade glioma in infant with rhabdoid tumor predisposition syndrome I (hereditary mutation in the SMARCB1 gene). The use of multimodal antitumor therapy according to the recommendations of the Medical University of Vienna (MUV ATRT) and metronomic chemotherapy was accompanied by a long-term effect. However, with the subsequent rapid progression of the disease, all treatment attempts were unsuccessful, and the patient died 44 months after diagnosis.
References
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