title: Treatment and Outcome in 65 Children with Optic Pathway Gliomas.
abstract: BACKGROUND
  Optic pathway gliomas (OPGs) 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 July 2007 and July 2014 were retrospectively reviewed. We evaluated the roles of surgical, ophthalmologic, endocrinologic, neurologic, and treatment aspects of care.
  
  
  RESULTS
  Sixty-five patients were included in this study, with a mean age of 5.3 years. OPGs 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 neurofibromatosis type 1. Four cases underwent surgical debulking, and 28 were biopsied (16 open, 11 stereotactic, and 1 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). Nonrepresentative sample (n = 1). The 4-year overall survival rate was 86.3% with mean follow-up period of 32.2 months.
  
  
  CONCLUSIONS
  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 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.
authors.affiliation: Department of Neurosurgery, Kasr Al-Ainy School of Medicine, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt. Electronic address: beltagy_mohamed@hotmail.com.
authors.forename: Mohamed A
authors.initials: MA
authors.lastname: El Beltagy
authors.affiliation: Department of Neurosurgery, Kasr Al-Ainy School of Medicine, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Reda
authors.affiliation: Department of Neurosurgery, Kasr Al-Ainy School of Medicine, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Abdelrhman
authors.initials: A
authors.lastname: Enayet
authors.affiliation: Department of Radiation Oncology, Children's Cancer Hospital, National Cancer Institute, Cairo University, Cairo, Egypt.
authors.forename: Mohamed Saad
authors.initials: MS
authors.lastname: Zaghloul
authors.affiliation: Consultant of Pediatric Oncology, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Madeha
authors.initials: M
authors.lastname: Awad
authors.affiliation: Consultant of Pediatric Oncology, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Wael
authors.initials: W
authors.lastname: Zekri
authors.affiliation: Department of Clinical Pathology, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Hala
authors.initials: H
authors.lastname: Taha
authors.affiliation: Department of Research, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
authors.forename: Nada
authors.initials: N
authors.lastname: El-Khateeb
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001921:Brain / Q000000981:diagnostic imaging / Q000187:drug effects / Q000601:surgery; D002675:Child, Preschool; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019635:Neurosurgical Procedures; D020339:Optic Nerve Glioma / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology / Q000628:therapy*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents
keywords: Chiasmal/hypothalamic glioma; Low-grade glioma; Neurofibromatosis; Optic pathway glioma