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Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy.

Rakotonjanahary J, De Carli E, Delion M, Kalifa C, Grill J, Doz F, Leblond P, Bertozzi AI, Rialland X, Brain Tumor Committee of SF - PLoS ONE (2015)

Bottom Line: The effect of potential risk factors on the risk of death was described using Cox regression analysis.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.Age and intracranial hypertension at diagnosis were significantly associated with a worse prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Oncology, University Hospital, Angers, France; INSERM CIE5 Robert Debre Hospital, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Sorbonne Paris Cité, Paris, France.

ABSTRACT

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

No MeSH data available.


Related in: MedlinePlus

Overall Survival for the whole population with optic pathway glioma.
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pone.0127676.g001: Overall Survival for the whole population with optic pathway glioma.

Mentions: Computed from the date of diagnosis, the 5- and 10-year OS rate were 95% [95% CI: 90.6–97.3] and 91.6% [95% CI: 86.5–94.8], respectively, for the whole population. Among the 79 patients who could have a follow-up of ≥ 15 years, the OS rate at 15 years was 80.7% [95% CI: 72.7–86.8], and among the 49 patients who could have a follow-up of ≥ 18 years, the OS rate at 18 years was 75.5 [95% CI: 65.6–83] (Fig 1).


Mortality in Children with Optic Pathway Glioma Treated with Up-Front BB-SFOP Chemotherapy.

Rakotonjanahary J, De Carli E, Delion M, Kalifa C, Grill J, Doz F, Leblond P, Bertozzi AI, Rialland X, Brain Tumor Committee of SF - PLoS ONE (2015)

Overall Survival for the whole population with optic pathway glioma.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4476571&req=5

pone.0127676.g001: Overall Survival for the whole population with optic pathway glioma.
Mentions: Computed from the date of diagnosis, the 5- and 10-year OS rate were 95% [95% CI: 90.6–97.3] and 91.6% [95% CI: 86.5–94.8], respectively, for the whole population. Among the 79 patients who could have a follow-up of ≥ 15 years, the OS rate at 15 years was 80.7% [95% CI: 72.7–86.8], and among the 49 patients who could have a follow-up of ≥ 18 years, the OS rate at 18 years was 75.5 [95% CI: 65.6–83] (Fig 1).

Bottom Line: The effect of potential risk factors on the risk of death was described using Cox regression analysis.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.Age and intracranial hypertension at diagnosis were significantly associated with a worse prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Oncology, University Hospital, Angers, France; INSERM CIE5 Robert Debre Hospital, Assistance Publique-Hôpitaux de Paris, University Paris Diderot, Sorbonne Paris Cité, Paris, France.

ABSTRACT

Background: 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.

Methods: 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.

Results: 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).

Conclusions: 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.

No MeSH data available.


Related in: MedlinePlus