Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy.
<h4>Background</h4>In terms of overall survival (OS), limited data are available for the very long-term outcomes of children treated for optic pathway glioma (OPG) with up-front chemotherapy. Therefore, we undertook this study with the aim of clarifying long-term OS and causes of death i...
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doaj-7090371360504479bf2e294e23959ef92021-03-04T07:59:15ZengPublic Library of Science (PLoS)PLoS ONE1932-62032015-01-01106e012767610.1371/journal.pone.0127676Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy.Josué RakotonjanaharyEmilie De CarliMatthieu DelionChantal KalifaJacques GrillFrançois DozPierre LeblondAnne-Isabelle BertozziXavier RiallandBrain Tumor Committee of SFCE<h4>Background</h4>In terms of overall survival (OS), limited data are available for the very long-term outcomes of children treated for optic pathway glioma (OPG) with up-front chemotherapy. Therefore, we undertook this study with the aim of clarifying long-term OS and causes of death in these patients.<h4>Methods</h4>We initiated and analyzed a historical cohort study of 180 children with OPG treated in France with BB-SFOP chemotherapy between 1990 and 2004. The survival distributions were estimated using Kaplan-Meier method. The effect of potential risk factors on the risk of death was described using Cox regression analysis.<h4>Results</h4>The OS was 95% [95% CI: 90.6-97.3] 5 years after diagnosis and significantly decreased over time without ever stabilizing: 91.6% at 10 years [95% CI: 86.5-94.8], 80.7% at 15 years [95% CI: 72.7-86.8] and 75.5% [95% CI: 65.6-83] at 18 years. Tumor progression was the most common cause of death (65%). Age and intracranial hypertension at diagnosis were significantly associated with a worse prognosis. Risk of death was increased by 3.1[95% CI: 1.5-6.2] (p=0.002) for patients less than 1 year old at diagnosis and by 5.2[95% CI: 1.5-17.6] (p=0.007) for patients with initial intracranial hypertension. Boys without diencephalic syndrome had a better prognosis (HR: 0.3 [95% CI: 0.1-0.8], p=0.007).<h4>Conclusions</h4>This study shows that i) in children with OPG, OS is not as favorable as previously described and ii) patients can be classified into 2 groups depending on risk factors (age, intracranial hypertension, sex and diencephalic syndrome) with an OS rate of 50.4% at 18 years [95% CI: 31.4-66.6] in children with the worst prognosis. These findings could justify, depending on the initial risk, a different therapeutic approach to this tumor with more aggressive treatment (especially chemotherapy) in patients with high risk factors.https://doi.org/10.1371/journal.pone.0127676 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Josué Rakotonjanahary Emilie De Carli Matthieu Delion Chantal Kalifa Jacques Grill François Doz Pierre Leblond Anne-Isabelle Bertozzi Xavier Rialland Brain Tumor Committee of SFCE |
spellingShingle |
Josué Rakotonjanahary Emilie De Carli Matthieu Delion Chantal Kalifa Jacques Grill François Doz Pierre Leblond Anne-Isabelle Bertozzi Xavier Rialland Brain Tumor Committee of SFCE Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. PLoS ONE |
author_facet |
Josué Rakotonjanahary Emilie De Carli Matthieu Delion Chantal Kalifa Jacques Grill François Doz Pierre Leblond Anne-Isabelle Bertozzi Xavier Rialland Brain Tumor Committee of SFCE |
author_sort |
Josué Rakotonjanahary |
title |
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. |
title_short |
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. |
title_full |
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. |
title_fullStr |
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. |
title_full_unstemmed |
Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy. |
title_sort |
mortality in children with optic pathway glioma treated with up-front bb-sfop chemotherapy. |
publisher |
Public Library of Science (PLoS) |
series |
PLoS ONE |
issn |
1932-6203 |
publishDate |
2015-01-01 |
description |
<h4>Background</h4>In terms of overall survival (OS), limited data are available for the very long-term outcomes of children treated for optic pathway glioma (OPG) with up-front chemotherapy. Therefore, we undertook this study with the aim of clarifying long-term OS and causes of death in these patients.<h4>Methods</h4>We initiated and analyzed a historical cohort study of 180 children with OPG treated in France with BB-SFOP chemotherapy between 1990 and 2004. The survival distributions were estimated using Kaplan-Meier method. The effect of potential risk factors on the risk of death was described using Cox regression analysis.<h4>Results</h4>The OS was 95% [95% CI: 90.6-97.3] 5 years after diagnosis and significantly decreased over time without ever stabilizing: 91.6% at 10 years [95% CI: 86.5-94.8], 80.7% at 15 years [95% CI: 72.7-86.8] and 75.5% [95% CI: 65.6-83] at 18 years. Tumor progression was the most common cause of death (65%). Age and intracranial hypertension at diagnosis were significantly associated with a worse prognosis. Risk of death was increased by 3.1[95% CI: 1.5-6.2] (p=0.002) for patients less than 1 year old at diagnosis and by 5.2[95% CI: 1.5-17.6] (p=0.007) for patients with initial intracranial hypertension. Boys without diencephalic syndrome had a better prognosis (HR: 0.3 [95% CI: 0.1-0.8], p=0.007).<h4>Conclusions</h4>This study shows that i) in children with OPG, OS is not as favorable as previously described and ii) patients can be classified into 2 groups depending on risk factors (age, intracranial hypertension, sex and diencephalic syndrome) with an OS rate of 50.4% at 18 years [95% CI: 31.4-66.6] in children with the worst prognosis. These findings could justify, depending on the initial risk, a different therapeutic approach to this tumor with more aggressive treatment (especially chemotherapy) in patients with high risk factors. |
url |
https://doi.org/10.1371/journal.pone.0127676 |
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