LG-70: DISSEMINATED LOW GRADE GLIOMA IN CHILDREN AND ADOLESCENTS- A RETROSPECTIVE REVIEW OF TREATMENT OUTCOME IN A SINGLE CENTRE

BACKGROUND: Low grade gliomas in children generally have an indolent clinical course and most children have a good outcome. Since disseminated disease is rare, there is a paucity of data on the incidence, clinical characteristics, tumour behaviour, and treatment outcome. METHODS: We conducted a retrospective analysis of children and adolescents with disseminated LGG at presentation or relapse treated at GOSH and UCH between 2003 and 2015. Data collected included: patient demographics, tumour histology, treatment modalities and outcomes both in terms of mortality, morbidity and functional deficits. RESULTS: Out of 295 children diagnosed with LGG during this period, 34 had disseminated disease either at diagnosis or recurrence; seven of these also had type 1 neurofibromatosis. Pilocytic astrocytoma was the predominant histological subtype and comprised 44% [n = 15] of the cohort. The most common location of the primary was the posterior fossa (29%). Twenty one patients [62%] had disseminated disease at diagnosis. Chemotherapy was the most common primary treatment modality [50%]. Treatment response after initial treatment was: stable disease or partial response - 59% [n = 20], progressive disease 41% [n = 14.] Median follow up for the cohort was 35 months [range, 1 day - 125 months]. Twenty four patients are currently alive, one was lost to follow up and nine have died. All but one death was due to disease progression. CONCLUSIONS: This confirms patients with disseminated low grade gliomas have poorer prognosis and survival. Although treatment modalities for disseminated LGG are multi-modal, prospective studies are needed to determine the true incidence and optimal treatment.