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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 according to the number of risk factors present at diagnosis: whole population (A), 0 or 1 risk factor (B), 2 or more risk factors (C).
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pone.0127676.g003: Overall survival according to the number of risk factors present at diagnosis: whole population (A), 0 or 1 risk factor (B), 2 or more risk factors (C).

Mentions: Using these factors associated with a poor prognosis (age, intracranial hypertension, sex and DS), we were able to describe 2 groups of patients: B (absence or presence of one of these poor prognosis factors); C (presence of 2 or more of these factors). There was a statistically significant difference in the 5, 10, 15 and 18-year OS between group B and C (HR: 4.5 [2.1–9.2], p<10−3). The OS rate at 18 years was 87.8% in the first group [95%CI: 78.4–93.3] compared to 50.4% in the second group [95%CI: 31.4–66.6]. These results are summarized in Fig 3.


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 according to the number of risk factors present at diagnosis: whole population (A), 0 or 1 risk factor (B), 2 or more risk factors (C).
© Copyright Policy
Related In: Results  -  Collection

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

pone.0127676.g003: Overall survival according to the number of risk factors present at diagnosis: whole population (A), 0 or 1 risk factor (B), 2 or more risk factors (C).
Mentions: Using these factors associated with a poor prognosis (age, intracranial hypertension, sex and DS), we were able to describe 2 groups of patients: B (absence or presence of one of these poor prognosis factors); C (presence of 2 or more of these factors). There was a statistically significant difference in the 5, 10, 15 and 18-year OS between group B and C (HR: 4.5 [2.1–9.2], p<10−3). The OS rate at 18 years was 87.8% in the first group [95%CI: 78.4–93.3] compared to 50.4% in the second group [95%CI: 31.4–66.6]. These results are summarized in Fig 3.

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