Current state and therapy strategies in paediatric low grade glioma

Over the last few decades, the paediatric neuro-oncologic community has gained a more comprehensive insight into paediatric low grade glioma. 10–20% of childhoods LGG associated with inherited disorders, mainly neurofibromatosis type 1 (NF1). The localisations of childhoods LGG are in decreasing order in the posterior fossa, the supratentorial midline (optic nerves, chiasm and hypothalamus), the cerebral hemispheres and the spinal cord. In many paediatric patients LGG is a disease curable by neurosurgical removal only. Survival data are favourable with a five year overall survival (OS) of 94.6% and progression free survival (PFS) of 69.4%. However, an important number of patients needs further chemotherapy or (in respect of patient age) radiotherapy. Treatment decisions will have to be made by an expert team of paediatric neuro-oncologists, neurosurgeons and radiation oncologists as part of international treatment protocols. The recent advances in biological studies on these tumours with identification of specific molecular changes may completely change our treatment approach in the future.