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Primary CNS tumors are relatively rare accounting for approximately 2-3% of all cancers. However, they comprise aggressive malignancies with a very poor prognosis and are responsible for 7% cancer deaths in the population younger than 70 years. The age distribution of CNS neoplasias is bimodal with a first peak in children, accounting for more than one fifth of all tumor diseases in children and a second one in adults between 45-70 years of age. In both groups surgical resection of tumors from vital brain structures constitutes a major clinical and ethical challenge, emphasizing the need for efficacious adjuvant therapies. Therefore, faithful mouse models which allow preclinical target validation are a major challenge for translational brain cancer research.

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