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Abstract Background Optic pathway gliomas (OPG) are rare tumors in children. Lesion extent, visual functions, neurofibromatosis 1 (NF1), and age are factors that guide treatment. This study evaluates the clinical characteristics, treatment, and outcome of children and adolescents with OPG treated over a 31‐year period in a single center. Methods Ninety‐five patients with OPG diagnosed between January 1990 and December 2021 were retrospectively evaluated. First‐line chemotherapy regimen consisted of vincristine and carboplatinum for 1 year. Radiotherapy was not used as first‐line treatment and tried to be avoided in the ones who progressed after first‐line treatment. Results Ninety‐five children (44 male, 51 female) with a median age of 52 (1–216) months were evaluated. Sixty‐three (66.3%) had NF1 and 10 (10,5%) diencephalic syndrome. The most common presenting symptoms were visual abnormalities and/or proptosis, nistagmus, and behavioral changes. Twenty‐one (22.1%) patients with NF1 had stable disease throughout the follow‐up period and received no treatment. Sixty‐three of 74 patients received treatment at diagnosis and 11 due to progression during follow‐up. Only one adolescent received radiotherapy at progression. Patients who progressed, received further line systemic treatment (vinblastine; bevacizumab; vincristine–cisplatinum–etoposide). Ten‐year overall survival in all patients, in patients with NF1, and without NF1 were 97.2%, 98%, and 95.8% ( p > .05), respectively; 10‐year progression‐free survival (PFS) in all patients, in patients with NF1, and without NF1 were 71.6%, 85.7%, and 54.2% ( p = .001), respectively. Conclusions In children with symptomatic/progressive OPG, chemotherapy consisting of vincristine–carboplatinum (VC) is effective. Radiotherapy may be avoided, especially in patients with NF1.
Kebudi et al. (Sun,) studied this question.