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LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes

INTRODUCTION: Whilst most patients with PLGG will survive, varying morbidities derived from patient,tumour & treatment characteristics can afflict life-long disabling functional impairments. No PLGG studies have evaluated potential prognostic factors for important functional outcomes. METHODS: W...

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Autores principales: Green, Katherine, Dahl, Christine, Jorgensen, Mette, O'Hare, Patricia, Opocher, Enrico, Slater, Olga, Gains, Jenny, Chang, Yen-Ching, Aquilina, Kristian, Bowman, Richard, Oldridge, Bronwen, Jacques, Thomas S, Stone, Thomas, Gan, Hoong-Wei, Bluebond-Langner, Myra, Hargrave, Darren
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9165037/
http://dx.doi.org/10.1093/neuonc/noac079.358
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author Green, Katherine
Dahl, Christine
Jorgensen, Mette
O'Hare, Patricia
Opocher, Enrico
Slater, Olga
Gains, Jenny
Chang, Yen-Ching
Aquilina, Kristian
Bowman, Richard
Oldridge, Bronwen
Jacques, Thomas S
Stone, Thomas
Gan, Hoong-Wei
Bluebond-Langner, Myra
Hargrave, Darren
author_facet Green, Katherine
Dahl, Christine
Jorgensen, Mette
O'Hare, Patricia
Opocher, Enrico
Slater, Olga
Gains, Jenny
Chang, Yen-Ching
Aquilina, Kristian
Bowman, Richard
Oldridge, Bronwen
Jacques, Thomas S
Stone, Thomas
Gan, Hoong-Wei
Bluebond-Langner, Myra
Hargrave, Darren
author_sort Green, Katherine
collection PubMed
description INTRODUCTION: Whilst most patients with PLGG will survive, varying morbidities derived from patient,tumour & treatment characteristics can afflict life-long disabling functional impairments. No PLGG studies have evaluated potential prognostic factors for important functional outcomes. METHODS: We performed retrospective analysis of all children diagnosed with PLGG at GOSH 1980-2021.Review of medical notes recorded patient demographics,tumour characteristics & treatment data.Functional outcomes included endocrine,educational,visual (in OPG),auditory & physical function.Multivariate regression analysis(p<0.05) examined associations between biological prognostic variables & functional outcomes. RESULTS: 814 patients were diagnosed with PLGG.731(90%) had 5-years follow-up from diagnosis & were included for functional analysis.Median age at diagnosis 7 years(0-17.9); 50.6%Male,12.2% NF1.Tumours were cerebral(27%),cerebellar(27%),hypothalamo-chiasmatic(19%),brainstem(7%),or other(20%);with disseminated disease in 5%.Pilocytic Astrocytoma constituted 46%.Molecular profiling of 133 revealed 5%BRAFV600E mutation,42%BRAF-KIAA1549 fusion.Treatments included: Surgery(70%),Chemotherapy(20%),& Radiotherapy(21%).20-year-OS 94%,PFS 76%;median follow-up 16 years(5-38). Documented neurocognitive deficiency(30%) associated with chemotherapy(HR2.36,95%CI 1.49-3.75,P<0.001), radiotherapy(HR 2.25,95%CI1.5-3.36,P<0.001) & male gender(HR 0.68,95%CI 0.49-0.95,P0.02)as independent poor-prognostic risk-factors.Chemotherapy(HR 5.7,95%CI1.4-22.3,P0.01) & radiotherapy(HR6.77,95%CI2.1-22.0,P0.001) were independent risk-factors for requirement of Educational-Health-Care-Plans(25%).9% attended specialised schools. Combined-limb-MRC-grade <18/20(6.4%) was independently-associated with receiving chemotherapy(HR 2.77,95%CI1.29-5.93,P0.01),& radiotherapy(HR 6.28,95%CI3.25-12.15,P<0.001).6% mobilised by wheelchair.Resolution of seizures occurred in 68% of 176 following PLGG treatment. Single/multiple endocrinopathies occurred in 9.3%/11%.Presence of 2+Endocrinopathies was associated with chemotherapy(HR6.82,95%CI4.0-14.4,P<0.001), radiotherapy(HR7.81,95%CI4.3-14.3,P<0.001),NF1(HR2.9,95%CI1.3-6,P0.01),OPGs(HR 1.3,95%CI1.2-1.5,P<0.001);with younger diagnostic-age(HR0.80,95%CI0.74-0.87,P<0.001) & initial surgical resection(HR0.3,95%CI0.15-0.7,P0.03) independent protective factors. Receiving chemotherapy/radiotherapy were independent prognostic-factors for Post-PLGG-treatment Brock grade 1+ hearing impairments(2.2%).Visual outcomes in 146 OPG patients:blindness in atleast 1 eye(4.8%),registered visual impairment(9.6%),& visual-aid use(6.2%). CONCLUSIONS: Whilst overall outcomes for PLGG are optimistic, some patients have significant functional impairments detrimental to quality-of-life.Further evaluation of longer-term functional outcomes and prognostic associations is justified.
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spelling pubmed-91650372022-06-05 LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes Green, Katherine Dahl, Christine Jorgensen, Mette O'Hare, Patricia Opocher, Enrico Slater, Olga Gains, Jenny Chang, Yen-Ching Aquilina, Kristian Bowman, Richard Oldridge, Bronwen Jacques, Thomas S Stone, Thomas Gan, Hoong-Wei Bluebond-Langner, Myra Hargrave, Darren Neuro Oncol Low Grade Glioma INTRODUCTION: Whilst most patients with PLGG will survive, varying morbidities derived from patient,tumour & treatment characteristics can afflict life-long disabling functional impairments. No PLGG studies have evaluated potential prognostic factors for important functional outcomes. METHODS: We performed retrospective analysis of all children diagnosed with PLGG at GOSH 1980-2021.Review of medical notes recorded patient demographics,tumour characteristics & treatment data.Functional outcomes included endocrine,educational,visual (in OPG),auditory & physical function.Multivariate regression analysis(p<0.05) examined associations between biological prognostic variables & functional outcomes. RESULTS: 814 patients were diagnosed with PLGG.731(90%) had 5-years follow-up from diagnosis & were included for functional analysis.Median age at diagnosis 7 years(0-17.9); 50.6%Male,12.2% NF1.Tumours were cerebral(27%),cerebellar(27%),hypothalamo-chiasmatic(19%),brainstem(7%),or other(20%);with disseminated disease in 5%.Pilocytic Astrocytoma constituted 46%.Molecular profiling of 133 revealed 5%BRAFV600E mutation,42%BRAF-KIAA1549 fusion.Treatments included: Surgery(70%),Chemotherapy(20%),& Radiotherapy(21%).20-year-OS 94%,PFS 76%;median follow-up 16 years(5-38). Documented neurocognitive deficiency(30%) associated with chemotherapy(HR2.36,95%CI 1.49-3.75,P<0.001), radiotherapy(HR 2.25,95%CI1.5-3.36,P<0.001) & male gender(HR 0.68,95%CI 0.49-0.95,P0.02)as independent poor-prognostic risk-factors.Chemotherapy(HR 5.7,95%CI1.4-22.3,P0.01) & radiotherapy(HR6.77,95%CI2.1-22.0,P0.001) were independent risk-factors for requirement of Educational-Health-Care-Plans(25%).9% attended specialised schools. Combined-limb-MRC-grade <18/20(6.4%) was independently-associated with receiving chemotherapy(HR 2.77,95%CI1.29-5.93,P0.01),& radiotherapy(HR 6.28,95%CI3.25-12.15,P<0.001).6% mobilised by wheelchair.Resolution of seizures occurred in 68% of 176 following PLGG treatment. Single/multiple endocrinopathies occurred in 9.3%/11%.Presence of 2+Endocrinopathies was associated with chemotherapy(HR6.82,95%CI4.0-14.4,P<0.001), radiotherapy(HR7.81,95%CI4.3-14.3,P<0.001),NF1(HR2.9,95%CI1.3-6,P0.01),OPGs(HR 1.3,95%CI1.2-1.5,P<0.001);with younger diagnostic-age(HR0.80,95%CI0.74-0.87,P<0.001) & initial surgical resection(HR0.3,95%CI0.15-0.7,P0.03) independent protective factors. Receiving chemotherapy/radiotherapy were independent prognostic-factors for Post-PLGG-treatment Brock grade 1+ hearing impairments(2.2%).Visual outcomes in 146 OPG patients:blindness in atleast 1 eye(4.8%),registered visual impairment(9.6%),& visual-aid use(6.2%). CONCLUSIONS: Whilst overall outcomes for PLGG are optimistic, some patients have significant functional impairments detrimental to quality-of-life.Further evaluation of longer-term functional outcomes and prognostic associations is justified. Oxford University Press 2022-06-03 /pmc/articles/PMC9165037/ http://dx.doi.org/10.1093/neuonc/noac079.358 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://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. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Low Grade Glioma
Green, Katherine
Dahl, Christine
Jorgensen, Mette
O'Hare, Patricia
Opocher, Enrico
Slater, Olga
Gains, Jenny
Chang, Yen-Ching
Aquilina, Kristian
Bowman, Richard
Oldridge, Bronwen
Jacques, Thomas S
Stone, Thomas
Gan, Hoong-Wei
Bluebond-Langner, Myra
Hargrave, Darren
LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title_full LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title_fullStr LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title_full_unstemmed LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title_short LGG-46. Survival Of The Fittest? A Prognostic Evaluation of Paediatric Low-Grade Glioma (PLGG) Survivor Functional Outcomes
title_sort lgg-46. survival of the fittest? a prognostic evaluation of paediatric low-grade glioma (plgg) survivor functional outcomes
topic Low Grade Glioma
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9165037/
http://dx.doi.org/10.1093/neuonc/noac079.358
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