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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 age at diagnosis (A), NF1 status (B), presence of DS (C) or intracranial hypertension (IH) (D) at diagnosis.
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pone.0127676.g002: Overall survival according to age at diagnosis (A), NF1 status (B), presence of DS (C) or intracranial hypertension (IH) (D) at diagnosis.

Mentions: We analyzed different clinical criteria that might influence the results: sex, age at diagnosis, NF1 associated with OPG, presence of DS or intracranial hypertension at diagnosis. The results are summarized in Fig 2.


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 age at diagnosis (A), NF1 status (B), presence of DS (C) or intracranial hypertension (IH) (D) at diagnosis.
© Copyright Policy
Related In: Results  -  Collection

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

pone.0127676.g002: Overall survival according to age at diagnosis (A), NF1 status (B), presence of DS (C) or intracranial hypertension (IH) (D) at diagnosis.
Mentions: We analyzed different clinical criteria that might influence the results: sex, age at diagnosis, NF1 associated with OPG, presence of DS or intracranial hypertension at diagnosis. The results are summarized in Fig 2.

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