Abstract Background and Objectives Pure optic nerve gliomas (PONG) are rare pediatric tumors typically managed conservatively to avoid disruption of the visual pathway. However, unlike chiasmatic optic pathway gliomas, PONGs frequently show limited response to chemotherapy or antiangiogenic agents, and the role of surgery remains controversial and non-routine. This study evaluates outcomes of upfront surgical resection in selected unilateral PONG without chiasmatic involvement, with particular focus on vision and ocular motility preservation. Methods We performed a retrospective analysis of 10 consecutive pediatric patients with unilateral PONG treated surgically at a single institution. Median age at diagnosis was 5 years (range 2–14), with a female predominance (6F/4M). All tumors were histologically confirmed pilocytic astrocytomas. Clinical presentation, prior treatments, surgical approach, pre- and postoperative visual function and ocular motility, complications, and follow-up were reviewed descriptively. Results Clinical presentation was dominated by proptosis (6/10) and/or acute visual loss (6/10), prompting surgical intervention. Baseline vision was severely compromised in 7/10 affected eyes, with variable ocular motility restriction. Complete resection was achieved in 9/10 patients, primarily via lateral orbitotomy. Three patients had received prior chemotherapy or bevacizumab with limited clinical benefit. Molecular data were limited, revealing isolated NF1 alterations, BRAF fusions, and FGFR alterations. Postoperative morbidity was minimal. Vision was preserved in all cases with patent vision at diagnosis. No ocular atrophy was observed in the long term, and ocular motility was preserved in all patients. No relapses were observed after a median follow-up of 6 years. Conclusions In selected unilateral PONG without chiasmatic involvement, surgical resection provides excellent long-term tumor control with preservation of ocular motility and maintenance of vision when present at diagnosis. These findings suggest that, in carefully selected cases, surgery may represent an effective, chemotherapy-sparing management strategy.
Solano-Paez et al. (Tue,) studied this question.