Identification of Novel Therapeutic Approaches for pHGGs

Gliomas are the most common central nervous system (CNS) tumors in children and adolescents, and they display a particularly broad range of clinical behavior. The impact of CNS tumors on infant mortality is very important. During the last decades there have been no advances in the prognosis of these tumors, while therapeutic strategies have changed very little. The majority of pediatric gliomas develop as benign, slow-growing lesions classified as grade I or II by the WHO classification of CNS tumors and among these, pilocytic astrocytoma accounts for ∼20% of all pediatric brain tumors. However, a substantial portion of gliomas develop over a short period of time and progress rapidly and are therefore classified as WHO grade III or IV high-grade gliomas (HGGs). Pediatric high-grade glioma (pHGG) is a devastating disease with a less than 20% survival rate 2 years after diagnosis, with the most aggressive forms being lethal within months.