Evaluation of Neurofibromatosis Type 1 Associated Optic Pathway Gliomas

Authors : Özge VURAL, Arzu OKUR, Faruk Güçlü PINARLI

Pages : 923-927

Doi:10.16899/jcm.1350153

View : 71 | Download : 41

Publication Date : 2023-09-30

Article Type : Research Paper

Abstract :Background/Aims: Optic pathway gliomas insert ignore into journalissuearticles values(OPGs); are low-grade gliomas histologically represented by pilocytic astrocytoma insert ignore into journalissuearticles values(PA); in 90% of cases, can develop from any part of the visual pathways such as optic nerve, chiasm, optic tract, or optic radiations which frequently involve the hypothalamus. OPGs account for 3–5% of childhood central nervous system insert ignore into journalissuearticles values(CNS); tumors and about 2% of pediatric glial lesions. OPGs are believed to be the most prevalent intracranial tumor in patients with neurofibromatosis type 1 insert ignore into journalissuearticles values(NF-1); and can occur in 15–20% of NF-1 cases. The aim of this study is to evaluate the clinical features and treatment response in patients diagnosed with optic glioma and NF-1. Methods: All cases diagnosed with OPG and received treatment in the Pediatric Oncology Department, between January 2015 to January 2021 were retrospectively evaluated. Inclusion criteria include children and adolescents with OPG aged between 0 and 18 years. The medical records insert ignore into journalissuearticles values(gender, age, tumor entity, tumor location); of patients, as well as their treatment history and magnetic resonance imaging insert ignore into journalissuearticles values(MRI); scans, were examined. The diagnosis of OPG was made clinically and radiologically by the tumor board. The recommendations of the Response Assessment in Pediatric Neuro-Oncology insert ignore into journalissuearticles values(RAPNO); working group were used in the diagnosis and evaluation of treatment response. Patients received intravenous chemotherapy with SIOP LGG 2004 insert ignore into journalissuearticles values(vincristine- carboplatin); with or without bevacizumab insert ignore into journalissuearticles values(10 mg/kg, started every 2 weeks);, therapy or vinblastine insert ignore into journalissuearticles values(3 mg/m2, weekly);. Results: This study included 27 cases during the study period from January 2015 to January 2021. In this study there were 14 male insert ignore into journalissuearticles values(51.8 %); and 13 female insert ignore into journalissuearticles values(48.1 %); patients. The median age was 4.8 insert ignore into journalissuearticles values(range: 0.5–14.9); years. Biopsy was performed in three patients and the diagnosis was low-grade glioma insert ignore into journalissuearticles values(pilocytic astrocytoma); for all of them. Chemotherapy was administered to 22 cases in total. Twelve patients received vincristine-carboplatine, 5 patients received vincristine-carboplatin with bevacizumab and 5 patients received vinorelbine. Radiological response was evaluated in all 22 patients at 3 months MRI. No patient had a radiological complete respons, 11 patients insert ignore into journalissuearticles values(50%); had partial response, 2 patients insert ignore into journalissuearticles values(9%); presented with a progressive disease, showing an increase in measurements of 35% and 9 patientsinsert ignore into journalissuearticles values(40.9%); had stable disease at the 3-month evaluation. Conclusions: Systemic and visual problems play a significant role in the selection of treatment for pediatric patients with optic gliomas. An essential treatment option for improving symptoms and reducing tumor size is systemic chemotherapy. A crucial therapy option for enhancing vision is bevacizumab for the patients with NF-associated OPG.
Keywords : optik yol gliomları, pediatrik kanserler, bevacizumab

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