European standard clinical practice recommendations for primary pediatric low-grade gliomas

Pediatric low-grade gliomas are the most common brain tumours in childhood and adolescence. Despite the excellent prognosis, pediatric low-grade glioma survivors may suffer from variable long-term complications and may require repeated therapies, implying that this is a chronic disease. The current review describes the European Standard Clinical Practice recommendations for low-grade gliomas at primary diagnosis, that were developed on behalf of SIOPe BTG LGG Working Group within the framework of European Reference Network PaedCan. The manuscript describes the diverse spectrum of pediatric low-grade gliomas in terms of location, age, underlying cancer predisposition syndromes, and special circumstances, such as infantile chiasmatic hypothalamic glioma and diencephalic syndrome, as well as current diagnostic criteria and indications for treatment. Furthermore, it provides current knowledge in histopathology and molecular pathology. Finally, the review focuses on the need for a multidisciplinary approach and treatment indications providing a guide on current treatment modalities, used as first-line therapy in Europe along with information on adverse effects, and follow-up.