Decoding optic pathway gliomas: Molecular insights and emerging therapies.

Area of Science:

• Neuro-oncology
• Pediatric Oncology
• Ophthalmology

Background:

• Optic Pathway Gliomas (OPGs) are low-grade astrocytic tumors affecting the visual pathway, predominantly in children.
• They represent 2% of cerebral gliomas, with 90% diagnosed before age 20, and are associated with neurofibromatosis type 1 (NF1).

Purpose of the Study:

• To review the classification, pathology, clinical presentation, and management of Optic Pathway Gliomas.
• To highlight the differences in prognosis and treatment approaches between pediatric and adult cases, and NF1-associated OPGs.

Main Methods:

• Literature review of OPGs, focusing on classification, clinical course, and therapeutic strategies.
• Analysis of diagnostic modalities including MRI and biopsy, and treatment options such as observation, surgery, chemotherapy, radiation, and targeted therapies.

Main Results:

• OPGs exhibit variable growth patterns influenced by BRAF alterations and NF1 status.
• Symptoms depend on tumor location; MRI is key for diagnosis.
• Pediatric cases generally have a better prognosis than adult cases, though visual impairment is frequent.

Conclusions:

• Treatment for OPGs is individualized, balancing efficacy with potential side effects, especially in young children.
• Emerging targeted therapies offer new hope for progressive cases.
• Further research, including randomized trials, is crucial for optimizing OPG management and improving long-term outcomes.