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Published on: November 11, 2021
Optic pathway glioma in children: 10 years of experience in a single institution
Dimitrios Doganis1, Apostolos Pourtsidis1, Kleonikos Tsakiris2
1a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
Insights
Optic pathway glioma (OPG) in children, often linked to neurofibromatosis type 1 (NF1), can be managed with chemotherapy. This treatment approach aids in preserving vision for most affected children.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Ophthalmology
Background:
- Optic pathway glioma (OPG) is a rare pediatric brain tumor.
- OPG is frequently associated with neurofibromatosis type 1 (NF1).
Purpose of the Study:
- To describe the characteristics, management, and outcomes of pediatric OPG patients.
- To evaluate the effectiveness of current treatment strategies for OPG.
Main Methods:
- Retrospective analysis of clinical charts from 2003-2013.
- Diagnosis confirmed via Magnetic Resonance Imaging (MRI).
- Review of treatment regimens and patient follow-up data.
Main Results:
- Fifteen of twenty patients had NF1; diagnosis in ten was during NF1 follow-up.
- Most patients received carboplatin-based chemotherapy.
- Six patients experienced vision or imaging deterioration, with some receiving further treatment (resection or radiation).
- No patients experienced total bilateral blindness.
Conclusions:
- Chemotherapy appears effective in preserving vision for most children with OPG.
- Early diagnosis, particularly during NF1 screening, is crucial.
- Multidisciplinary management is essential for optimal OPG outcomes.
Abstract:
Optic pathway glioma (OPG) is a rare brain tumor that occurs more commonly during early childhood and is frequently associated with neurofibromatosis type 1 (NF1). In this study, our aim was to describe the characteristics, management, and outcome of patients with OPG. We retrospectively analyzed the clinical charts of all children diagnosed with OPG at our institution from 2003 to 2013. Twenty children (11 boys and 9 girls, median age: 5 and 3/12 years; NF1: 15/20) were diagnosed with OPG. The diagnosis was based on magnetic resonance imaging (MRI) findings. A biopsy was useful in 3 patients. The main reason for seeking medical advice was decreased vision (7/20 patients), whereas in 10/20 patients, the diagnosis was established during the routine follow-up for their NF1. Fifteen patients demonstrated MRI findings of optic nerve involvement and/or chiasmal tumor, whereas in 5 children, postchiasmal structures were also involved. Sixteen patients (16/20) received carboplatin-based regimens, whereas 4/20 patients were only under close observation. Six patients showed deterioration of visual acuity and/or imaging findings at the end of treatment and/or during their follow-up. Three of them (3/6) underwent tumor resection, whereas 1 (1/6) received radiation treatment. None of our patients had total blindness from both eyes. Half of our patients were diagnosed during follow-up for their NF1, the incidence of which was high in our group. Our data suggest that chemotherapy helps in the preservation of vision in the majority of children.
