Treatment and Outcome in 65 Children with Optic Pathway Gliomas

• Published in: World neurosurgery 2016-05, Vol.89, p.525-534
• Main Authors: El Beltagy, Mohamed A., MD, Reda, Mohamed, MD, Enayet, Abdelrhman, M.SC, Zaghloul, Mohamed Saad, MD, Awad, Madeha, MD, Zekri, Wael, MD, Taha, Hala, MD, El-Khateeb, Nada
• Format: Article
• Language: English
• Subjects: Antineoplastic Agents - therapeutic use; Brain - diagnostic imaging; Brain - drug effects; Brain - surgery; Chiasmal/hypothalamic glioma; Child, Preschool; Disease Management; Female; Follow-Up Studies; Humans; Low-grade glioma; Magnetic Resonance Imaging; Male; Neurofibromatosis; Neurosurgery; Neurosurgical Procedures; Optic Nerve Glioma - diagnostic imaging; Optic Nerve Glioma - pathology; Optic Nerve Glioma - physiopathology; Optic Nerve Glioma - therapy; Optic pathway glioma; Retrospective Studies; Survival Analysis; Treatment Outcome
• ISSN: 1878-8750; 1878-8769
• DOI: 10.1016/j.wneu.2016.02.042

Abstract Introduction Optic pathway gliomas (OPG) are rare neoplasms in children with an unpredictable clinical course. There is significant controversy regarding the optimal management and outcome of these patients. Methods Charts of all patients with OPG diagnosed and treated at Children’s Cancer Hospital Egypt between August 2007 and December 2013 were retrospectively reviewed. Evaluation of the role of surgical, ophthalmological, endocrinological, neurological and treatment aspects of care were reviewed. Results 65 patients were included in this study, with a mean age of 5.3 years. OPG were chiasmatic (n=25), optic nerve (n=18), hypothalamic (n=7) and chiasmatic/ hypothalamic (n=7). Extensive involvement of the optic pathway was seen in an additional 8 patients. Twenty cases had NF1. Four cases underwent surgical debulking and 28 were biopsied (16 open, 11 stereotactic and one endoscopic). Nine of the 18 optic nerve tumors were managed by total excision. Twenty four patients did not undergo any surgical intervention. Forty five patients received chemotherapy. Histopathology revealed pilocytic (n=20), pilomyxoid (n=15), fibrillary astrocytoma (n=4), and grade I papillary-glioneuronal tumor (n=1). Non representative sample (n=1). The 4 years overall survival rate was 86.3% with mean follow-up period of 32.2 months. Conclusion The initial role of surgery in newly developed OPG is biopsy for tissue diagnosis and relief of the hydrocephalus if present, followed by chemotherapy. Chemotherapy decreases or stabilizes the tumor size in most of the cases, leading to preservation of both visual and endocrinal functions. The most significant prognostic factor confirmed in this study was the age of the patient.