Paediatric-type low-grade diffuse gliomas

General

  • Paediatric-type tumors may sometimes occur in adults, particularly young adults, and adult-type tumours may more rarely occur in children
  • Overall, paediatric low-grade gliomas are the most frequent brain tumours in children accounting for approximately 30% of all cases with circumscribed gliomas (discussed below) being far more common than diffuse low-grade gliomas
  • The need to separate adult and paediatric diffuse low grade gliomas is evident given the differing genetic landscape and the more aggressive clinical course of low-grade gliomas in adults, where they have a higher propensity for malignant transformation
  • The category of paediatric type diffuse low-grade glioma was introduced to distinguish tumours driven by an activation in the mitogen-activated protein kinase (MAPK) pathway from adult-type low-grade gliomas which are usually IDH driven

Consist of