Abstract

Pediatric central nervous system (CNS) tumors differ in their relative frequency, location, histology, biological behavior and prognosis from tumors in adults. Accurate neuropathological classification of CNS tumors is essential for therapeutic decisions and inclusion in therapy optimization studies. Tissue samples are analyzed by standardized conventional histological, immunohistological and molecular pathological methods and diagnosed according to the current World Health Organization (WHO) classification for CNS tumors (2021). By identifying characteristic genetic alterations and specific epigenetic signatures, the precision in the classification of pediatric brain tumors has significantly improved in recent years. The WHO classification allows aworldwide uniform, standardized classification of brain tumors and forms the basis for comparability of international epidemiological and clinical data. In some tumor types, such as childhood gliomas and embryonal tumors, key molecules and signaling pathways have been identified in recent years that represent starting points for new mechanism-based therapeutic modalities in the treatment of these patients.

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