Treatment of isolated pediatric optic nerve glioma: A nationwide retrospective cohort study and systematic literature review on visual and radiological outcome

Carlien A Bennebroek ^1,2, Maartje C Montauban van Swijndregt ¹, Judith van Zwol ¹

¹Orbital Center, Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
²Cancer Center Amsterdam, Cancer Treatment and Quality of Life, Amsterdam, The Netherlands.
³Department of Radiology and Nuclear Medicine, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.
⁴Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
⁵ENCORE-NF1 Center, Department of General Pediatrics, Erasmus MC, Rotterdam, The Netherlands.
⁶Department of Ophthalmology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁷Department of Radiology and Nuclear Medicine, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
⁸Department of Neuro-Oncology, Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands.
⁹Department of Ophthalmology, University Medical Center Utrecht, Utrecht, The Netherlands.
¹⁰Cancer Center Amsterdam, Imaging and Biomarkers, Amsterdam, The Netherlands.

⁰Orbital Center, Department of Ophthalmology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Pediatric Blood & Cancer +

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October 9, 2024

View abstract on PubMed

Summary

Systemic anticancer therapy (SAT) is a preferred treatment for progressive optic nerve glioma (ONG) in children, potentially preserving vision. Surgery may reduce proptosis in blind eyes, while radiotherapy requires caution due to vision loss risks.

Area Of Science

Pediatric Oncology
Ophthalmology
Neuro-oncology

Background

Optic nerve glioma (ONG) is a rare pediatric malignancy.
Lack of global treatment consensus for progressive ONG.

Purpose Of The Study

Investigate treatment strategies for pediatric ONG.
Assess impact of systemic anticancer therapy (SAT), surgery, and radiotherapy on visual acuity and tumor volume.

Main Methods

Retrospective multicenter cohort study (1995-2020).
Evaluated treatment responses and outcomes for 21 ONGs in 20 children.
Primary endpoints: best-corrected visual acuity (BCVA) and tumor volume (TV) changes.

Main Results

SAT stabilized or improved BCVA in 66.6% and reduced TV by 45.1% in 13 patients.
Surgery led to blindness in all four treated eyes.
Radiotherapy worsened BCVA in 66.7% and initially increased TV.

Conclusions

SAT is a preferred modality for progressive ONG, offering potential visual rescue.
Complete resection effective for proptosis in pre-blind eyes.
Radiotherapy use demands careful consideration due to severe visual impairment risks.

Keywords:

MRI children low grade glioma optic nerve glioma response assessment visual function