Abstract
Central nervous system (CNS) embryonal tumors are devastating cancers in children, consisting of medulloblastomas, CNS primitive neuroectodermal tumors, and atypical teratoid/rhabdoid tumors. One of the reasons that CNS embryonal tumors remain difficult to treat is their rarity, which makes conducting clinical trials for these tumors difficult. Recent advances of molecular biology have led us to identify molecular and genetic causality of brain tumors. Based on the genetic alterations found in humans, multiple models of human CNS embryonal tumors have been generated in genetically engineered mice. These mouse models are valuable tools for understanding brain tumor biology and discovering novel therapeutic targets and drugs. In this article, we review molecular and cytogenetic characteristics of human CNS embryonal tumors and corresponding mouse models that have been developed. These findings indicate that common genetic abnormalities are seen in variants of human CNS embryonal tumors, and multiple histological variants of these tumors can be generated from a single set of genetic abnormalities in mice. These data provide insight into the biology and classification of CNS embryonal tumors.
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