Abstract
BACKGROUND: Low grade gliomas (LGG) are the most frequent tumors of the central nervous system in children. Pediatric LGG are a heterogeneous set of tumors. They encompass tumors of astrocytic, oligodendroglial, and mixed glial-neuronal histology, and are classified according to the World Health Organization (WHO). METHODS: The objective of our study was to analyze the outcome of patients with LGG from the Department of Pediatric Hematology and Oncology in Bratislava, Slovakia treated in period from January 2000 up to December 2015. The cohort consisted of 139 patients with LGG, 83 boys/56 girls. RESULTS: The most common histological type was pilocytic astrocytoma (WHO grade I) in 64 children. Primary surgery was performed in 102 children and 72 from these do not need any further therapy. Forty eight patients received chemotherapy first or other line therapy. Radiotherapy was used 26 times. In our study median follow up was 82,3 months and overall survival for the whole cohort was 97,2%. CONCLUSION: In pediatric low grade glioma surgery is the mainstay of therapy. Ten year overall survival rates for patients whose tumors are completely resected are 90% or greater. Chemotherapy is reserved for progressive or recurrent tumors. Radiotherapy can also improve OS but is associated with more frequent adverse effects. Recent advent of integrated genomics and NGS has implicated the activation of RAS/RAF/MEK pathway in tumor genesis of LGG. Many of these biological advances are changing treatment paradigma, particularly in low-grade gliomas, where rationale targeted therapies are currently being explored in clinical trials.