SURVIVAL OUTCOMES AND PROGNOSTIC FACTORS OF LOW-INCIDENCE HIGH-GRADE CNS MALIGNANCY IN CHILDREN: A SINGLE-CENTRE STUDY

AIM: The aims of this study are to summarise and quantify survival outcomes in the paediatric high-grade central nervous system (CNS) malignancy population with low-incidence tumours at the Sydney Children’s Hospital, and identify key variables that influence survival outcomes. A retrospective review was conducted to identify patients aged ≤18years at the Sydney Children’s Hospital treated at the Department of Radiation Oncology between April 1992 and April 2016, identifying thirty-five eligible patients with pathology-confirmed high-grade disease. Overall survival (OS) and progression-free survival (PFS) were evaluated to determine the impact of presenting clinical features and treatment on survival outcomes. The full cohort had a median OS of 16.7 months and a median PFS of 8.8 months. Histological subtype, headache, negative glial fibrillary acidic protein (GFAP) immunochemistry, gross total resection, definitive radiation therapy, radiation dose of 5000cGy-5999cGy, and definitive chemotherapy were found to improve OS on univariate analysis (p<0.05). Histological subtype, headache and nil limb signs were also found to improve PFS on univariate analysis (p<0.05). Multivariate analysis identified surgical resection, histological subtype and presence of headache at presentation as independent prognosticators of OS. Survival outcomes at the Sydney Children’s Hospital are comparable to the literature for PFS, but OS outcomes are below what would be expected in this population. Observed survival outcomes and clinical prognosticators need to be validated through studies with larger samples to produce more robust findings and ensure the external validity of the conclusions obtained.