[Optic nerve glioma in children].

Area of Science:

• Pediatric Oncology
• Neuro-oncology
• Ophthalmology

Background:

• Optic pathway gliomas (OPGs) are rare pediatric brain tumors, comprising 3-5% of childhood brain tumors and 4% of orbital tumors.
• A significant association exists between OPGs and Neurofibromatosis type 1 (NF 1), with an estimated incidence of 30-58% in OPG patients.
• These tumors are typically pilocytic astrocytomas, and regression has been observed, especially in patients with NF 1.

Purpose of the Study:

• To summarize the current understanding and management strategies for optic pathway gliomas in children.
• To highlight the diagnostic modalities and treatment options based on tumor characteristics and patient presentation.

Main Methods:

• Review of existing literature on optic pathway gliomas.
• Discussion of diagnostic imaging techniques, including CT and MRI.
• Analysis of surgical, chemotherapeutic, and radiotherapeutic treatment approaches.

Main Results:

• Exophthalmos and vision loss are common presenting symptoms of progressive OPGs.
• Complete surgical removal offers a cure, but is reserved for severe cases with significant visual impairment.
• Chemotherapy is a primary treatment for growing tumors with useful vision confined to the orbit.

Conclusions:

• Management of OPGs requires a tailored approach based on tumor extent, growth, and visual function.
• The role of radiotherapy, particularly proton beam therapy, for intraorbital gliomas requires further investigation.