Long-term clinical and visual outcomes after surgical resection of pediatric pilocytic/pilomyxoid optic pathway gliomas

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OBJECTIVE

The choice of treatment modality for optic pathway gliomas (OPGs) is controversial. Chemotherapy is widely regarded as first-line therapy; however, subtotal resections have been reported for decompression or salvage therapy as first- and second-line treatment. The goal of this study was to further investigate the role and efficacy of resection for OPGs.

METHODS

A retrospective chart review was performed on 83 children who underwent surgical treatment for OPGs between 1986 and 2014. Pathology was reviewed by a neuropathologist. Clinical outcomes, including progression-free survival (PFS), overall survival (OS), and complications, were analyzed.

RESULTS

The 5- and 10-year PFS rates were 55% and 46%, respectively. The 5- and 10-year OS rates were 87% and 78%, respectively. The median extent of resection was 80% (range 30%–98%). Age less than 2 years at surgery and pilomyxoid features of the tumor were found to be associated with significantly lower 5-year OS. No difference was seen in PFS or OS of children treated with surgery as a first-line treatment compared with children with surgery as a second- or third-line treatment. Severe complications included new disabling visual deficit in 5%, focal neurological deficit in 8%, and infection in 2%. New hormone deficiency occurred in 22% of the children.

CONCLUSIONS

Approximately half of all children experience a long-term benefit from resection both as primary treatment and as a second-line therapy after failure of primary treatment. Primary surgery does not appear to have a significant benefit for children younger than 2 years or tumors with pilomyxoid features. Given the risks associated with surgery, an interdisciplinary approach is needed to tailor the treatment plan to the individual characteristics of each child.

ABBREVIATIONS NF1 = neurofibromatosis type 1; OPG = optic pathway glioma; OS = overall survival; PA = pilocytic astrocytoma; PA/PMXA = PA with pilomyxoid features; PFS = progression-free survival; PMXA = pilomyxoid astrocytoma; STR = subtotal resection.

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Article Information

Correspondence Eveline Teresa Hidalgo: The Hassenfeld Children’s Hospital at NYU Langone Health, New York, NY. teresa.hidalgo@nyumc.org.

INCLUDE WHEN CITING Published online May 17, 2019; DOI: 10.3171/2019.2.PEDS18529.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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Figures

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    Patients were grouped by treatment pathway. Patients with surgery as first-line treatment without adjuvant treatment comprised group 1, and those with surgery as second- or third-line treatment comprised group 2. Figure is available in color online only.

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    Kaplan-Meier curves showing PFS and OS in treatment groups 1 and 2. There were no significant differences in 20-year PFS (p = 0.8; upper) and 20-year OS (p = 0.8; lower). Figure is available in color online only.

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    Kaplan-Meier curves showing PFS and OS in children younger than 2 years and those at least 2 years old at the time of surgery. Children younger than 2 years at the time of surgery had worse PFS and OS compared to children 2 years and older, with a tendency toward difference in 20-year PFS (p = 0.06; upper) and a significant difference (*) in 20-year OS (p = 0.03; lower). Figure is available in color online only.

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    Kaplan-Meier curves showing PFS (upper) and OS (lower) overall in children with PAs compared with those in the pilomyxoid group (PMXA or PA/PMXA). Figure is available in color online only.

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    Visual status in 65 patients at last follow-up compared with preoperative visual status. Figure is available in color online only.

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