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Disseminated Low Grade Glioma in Children and Young Adults

Bell, R; Kirkwood, A; Hargrave, D; Michalski, A; Hyare, H; Jacques, T; Stoneham, S; ... Shankar, A; + view all (2020) Disseminated Low Grade Glioma in Children and Young Adults. ARC Journal of Cancer Science , 6 (1) pp. 7-18. 10.20431/2455-6009.0601002. Green open access

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Abstract

Background: Disseminated low-grade gliomas [d-LGG] in children, teenagers and young adults [TYA] are rare and the treatment outcome is worse than for than for those with localised disease. We present a retrospective institutional and ethical review board approved report of d-LGG in children and TYA treated at the London Cancer Paediatric & Adolescent Neuro-Oncology Service. Purpose: Between 1998 and 2014, 36 patients with d-LGG either at diagnosis or during follow up were identified. Anonymized data were retrospectively collected from the hospital databases and included patient demographics, modality of diagnosis, treatment received, recurrences or disease progression and outcome. Results: The median age of our cohort was 4.5 years and median follow up was 6 years. 60% had disseminated disease at diagnosis; pilocytic astrocytoma was the commonest histological subtype (n=18, 50%). Chemotherapy was the most common treatment modality utilised. The 6-year overall survival for those with localised disease with dissemination during follow-up and disseminated disease at presentation were 77.9% and 76.1% respectively. Progression free survival at 1, 2 and 3 years were 64.3%, 50% and 21.4% and 57.3, 43% and 38.2% respectively. Conclusion: Children with d-LGG treated have a poor outcome. A significant proportion of patients have multiple recurrences or disease progressions. There appears to be no difference in OS in those who had disseminated disease at diagnosis compared to those who developed disseminated disease at a later time point during follow up. Prospective international studies with molecular genetic profiling will help clarify the best treatment approach for this group of patients.

Type: Article
Title: Disseminated Low Grade Glioma in Children and Young Adults
Open access status: An open access version is available from UCL Discovery
DOI: 10.20431/2455-6009.0601002
Publisher version: https://doi.org/10.20431/2455-6009.0601002
Language: English
Additional information: Copyright: © 2020 Authors. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Oncology
URI: https://discovery.ucl.ac.uk/id/eprint/10182955
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