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Isolated optic nerve gliomas: a multicenter historical cohort study.
Ben Shofty1,2, Liat Ben-Sira1,3, Anat Kesler1,4
11The Gilbert Israeli Neurofibromatosis Center, Dana Children's Hospital.
Journal of Neurosurgery. Pediatrics
|October 7, 2017
Summary
Isolated optic nerve gliomas (IONGs) are dynamic tumors with high progression rates. Early chemotherapy consideration may improve outcomes in this rare OPG subgroup.
Area of Science:
- Neuro-oncology
- Ophthalmology
- Pediatric Neurology
Background:
- Isolated optic nerve gliomas (IONGs) are rare optic pathway gliomas (OPGs).
- Limited data exists on IONG natural history and optimal management.
- Current management mirrors that of other OPGs due to lack of specific evidence.
Purpose of the Study:
- To determine the natural history of isolated optic nerve gliomas.
- To analyze tumor behavior, visual outcomes, and treatment responses in IONGs.
Main Methods:
- Multicenter retrospective cohort study.
- Inclusion criteria: glioma of optic nerve only, ≥1 year follow-up, ≥2 MRI, ≥2 neuro-ophthalmological exams.
- 36 patients (39 tumors) analyzed, with a mean follow-up of 5.6 years.
Main Results:
- 59% of IONGs progressed; 18% showed spontaneous regression (all in neurofibromatosis Type 1).
- 51% of patients experienced visual decline, with 90% of those showing further deterioration.
- Chemotherapy improved vision in 55% (5/9) of treated patients; surgery showed no recurrence.
Conclusions:
- IONGs exhibit high radiological progression and visual deterioration rates compared to general OPG populations.
- Chemotherapy may offer superior response rates in IONGs, warranting early consideration.
- Further research into IONG-specific management strategies is crucial.
