Doganis Dimitrios, Pourtsidis Apostolos, Tsakiris Kleonikos, Baka Margarita, Kouri Agathi, Bouhoutsou Despina, Varvoutsi Maria, Servitzoglou Marina, Dana Helen, Kosmidis Helen
a Department of Oncology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
b Department of Ophthalmology , P. & A. Kyriakou Children's Hospital , Athens , Greece.
Pediatr Hematol Oncol. 2016 Mar;33(2):102-8. doi: 10.3109/08880018.2016.1155101. Epub 2016 Mar 23.
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.
视路胶质瘤（OPG）是一种罕见的脑肿瘤，在儿童早期更为常见，且常与1型神经纤维瘤病（NF1）相关。在本研究中，我们的目的是描述OPG患者的特征、治疗及预后情况。我们回顾性分析了2003年至2013年在我院诊断为OPG的所有儿童的临床病历。20名儿童（11名男孩和9名女孩，中位年龄：5岁3个月；NF1：15/20）被诊断为OPG。诊断基于磁共振成像（MRI）结果。3例患者进行了活检。就医的主要原因是视力下降（7/20例患者），而10/20例患者是在NF1的常规随访中确诊的。15例患者MRI显示视神经受累和/或视交叉肿瘤，而5例儿童视交叉后结构也受累。16例患者（16/20）接受了以卡铂为基础的治疗方案，而4/20例患者仅接受密切观察。6例患者在治疗结束时和/或随访期间视力和/或影像学表现恶化。其中3例（3/6）接受了肿瘤切除术，1例（1/6）接受了放射治疗。我们的患者均未出现双眼完全失明。我们一半的患者是在NF1随访期间确诊的，在我们的研究组中其发病率较高。我们的数据表明，化疗有助于大多数儿童保留视力。
