ABSTRACT
High-grade glioma (HGG), the most frequently encountered malignant primary brain tumor in adult neuro-oncological practice, is relatively rare in pediatric practice, accounting for only around 8–12% of primary central nervous system (CNS) tumors in this age group, 1 and remains a significant challenge for management. Children present with symptoms of raised intracranial pressure or focal deficits with a rapidly deteriorating course. The diagnosis will typically be suspected on imaging with an inhomogeneously enhancing and infiltrative tumor and will be confirmed at either biopsy or debulking surgery. Maximal safe surgery with focal radiotherapy and chemotherapy is the standard of care but the prognosis with this remains bleak, with relapse within 1 or 2 years the rule and 3-year survival only around 20% in recent series. 2 Biological, molecular, and genetic studies indicate that these tumors are distinct from their counterparts in adults, 3 and it is hoped that enhanced understanding of tumor biology and immunology may hold the key to novel targeted agents which improve survival in the years to come.
