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NF1 optic pathway glioma. Analysing risk factors for visual outcome and indications to treat

Azizi, AA; Walker, DA; Liu, J-F; Sehested, A; Jaspan, T; Pemp, B; Simmons, I; ... SIOPE NF1 OPG Nottingham, UK, Workshop 2014; + view all (2020) NF1 optic pathway glioma. Analysing risk factors for visual outcome and indications to treat. Neuro-Oncology 10.1093/neuonc/noaa153. (In press). Green open access

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Abstract

Background: The aim of the project was to identify risk factors associated with visual progression and treatment indications in pediatric patients with Neurofibromatosis type 1 associated optic pathway gliomas (NF1-OPG). Methods: A multi-disciplinary expert group consisting of ophthalmologists, pediatric neuro-oncologists, neurofibromatosis specialists and neuro-radiologists involved in therapy trials assembled a cohort of children with NF1-OPG from six European countries with complete clinical, imaging and visual outcome datasets. Using methods developed during a consensus workshop, visual and imaging data were reviewed by the expert team and analyzed to identify associations between factors at diagnosis with visual and imaging outcomes. Results: 83 patients (37 males, 46 females, mean age 5.1±2.6 years; 1-13.1 years) registered in the European treatment-trial SIOP LGG-2004 (recruited 2004-2012) were included. They were either observed or treated (at diagnosis/ after follow-up). In multivariable analysis, factors present at diagnosis associated with adverse visual outcomes included: multiple visual signs and symptoms (adjOR 8.33, 95%CI 1.9-36.45); abnormal visual behavior (adjOR 4.15, 95%CI 1.20-14.34); new onset of visual symptoms (adjOR 4.04, 95%CI 1.26-12.95) and optic atrophy (adjOR 3.73, 95%CI 1.13-12.53). Squint, posterior visual pathway tumor involvement, and bilateral pathway tumor involvement, showed borderline significance. Treatment appeared to reduce tumor size but improved vision in only 10/45 treated patients. Children with visual deterioration after primary observation are more likely to improve with treatment than children treated at diagnosis. Conclusions: The analysis identified the importance of symptomatology, optic atrophy and history of vision loss as predictive factors for poor visual outcomes in children with NF1-OPG.

Type: Article
Title: NF1 optic pathway glioma. Analysing risk factors for visual outcome and indications to treat
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/neuonc/noaa153
Publisher version: https://doi.org/10.1093/neuonc/noaa153
Language: English
Additional information: This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Neurofibromatosis type 1, optic glioma, risk assessment, vision, visual acuity
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 Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Biology and Cancer Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10106455
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