Background: Optic pathway gliomas (OPGs) are rare tumors predominantly affecting children and are often associated with neurofibromatosis type 1 (NF1). Their variable clinical course and critical visual and neuroendocrine pathway involvement present significant management challenges. This systematic review aims to evaluate the role of surgical intervention in OPGs, focusing on its impact on visual outcomes, postoperative tumor status, and treatment-related complications.
Methods: A systematic review was conducted following PRISMA guidelines. Data were extracted from peer-reviewed studies reporting surgical outcomes in OPG patients, including Dodge classification, type of surgical approach, intervention details, visual outcomes, tumor progression, progression-free survival (PFS), overall survival (OS), and complications.
Results: A total of 13 studies comprising 661 patients were included. Dodge Type III tumors were the most commonly reported. Surgical interventions included biopsy, subtotal resections, gross total resection (GTR), and debulking. Visual outcomes were variable; visual improvement was observed in a minority of cases, stable vision was the most commonly reported outcome, and others documented visual deterioration. Tumor status after surgery was stable in a majority of patients (up to 62,8%). Reported PFS and OS show 5-year OS rates ranging from 84.1% to 97.7% and PFS rates from 47.7% to 70.6%, indicating high survival with moderate variability in disease progression. Reported complications included visual loss, endocrine dysfunction, shunt failure, and mortality in a small subset.
Conclusion: Surgical intervention in OPGs is mainly diagnostic or decompressing. Visual improvement is uncommon; stability is more frequent. Aggressive surgery risks deterioration, highlighting the need for careful planning and standardized studies to guide management.
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Review Article |
Subject:
Cancer Surgery Received: 2025/05/26 | Accepted: 2025/06/13 | Published: 2025/06/30