title: Optic pathway glioma in children: 10 years of experience in a single institution.
abstract: Optic pathway glioma (OPG) is a rare brain tumor that occurs more commonly during early childhood and is frequently associated with neurofibromatosis type 1 (NF1). In this study, our aim was to describe the characteristics, management, and outcome of patients with OPG. We retrospectively analyzed the clinical charts of all children diagnosed with OPG at our institution from 2003 to 2013. Twenty children (11 boys and 9 girls, median age: 5 and 3/12 years; NF1: 15/20) were diagnosed with OPG. The diagnosis was based on magnetic resonance imaging (MRI) findings. A biopsy was useful in 3 patients. The main reason for seeking medical advice was decreased vision (7/20 patients), whereas in 10/20 patients, the diagnosis was established during the routine follow-up for their NF1. Fifteen patients demonstrated MRI findings of optic nerve involvement and/or chiasmal tumor, whereas in 5 children, postchiasmal structures were also involved. Sixteen patients (16/20) received carboplatin-based regimens, whereas 4/20 patients were only under close observation. Six patients showed deterioration of visual acuity and/or imaging findings at the end of treatment and/or during their follow-up. Three of them (3/6) underwent tumor resection, whereas 1 (1/6) received radiation treatment. None of our patients had total blindness from both eyes. Half of our patients were diagnosed during follow-up for their NF1, the incidence of which was high in our group. Our data suggest that chemotherapy helps in the preservation of vision in the majority of children.
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Doganis
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Apostolos
authors.initials: A
authors.lastname: Pourtsidis
authors.affiliation: b Department of Ophthalmology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Kleonikos
authors.initials: K
authors.lastname: Tsakiris
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Margarita
authors.initials: M
authors.lastname: Baka
authors.affiliation: b Department of Ophthalmology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Agathi
authors.initials: A
authors.lastname: Kouri
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Despina
authors.initials: D
authors.lastname: Bouhoutsou
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Maria
authors.initials: M
authors.lastname: Varvoutsi
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Marina
authors.initials: M
authors.lastname: Servitzoglou
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Helen
authors.initials: H
authors.lastname: Dana
authors.affiliation: a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
authors.forename: Helen
authors.initials: H
authors.lastname: Kosmidis
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000453:epidemiology; D020339:Optic Nerve Glioma / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000503:physiopathology; D012189:Retrospective Studies; D014792:Visual Acuity
keywords: Children; glioma; optic pathway