title: Optic glioma in children: a retrospective analysis of 101 cases.
abstract: OBJECTIVE
  To evaluate the clinical characteristics and long-term outcome of pediatric patients with optic glioma.
  
  
  METHODS
  A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease.
  
  
  RESULTS
  The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease.
  
  
  CONCLUSIONS
  Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies.
authors.affiliation: Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey. hupog@tr.net
authors.forename: Ali
authors.initials: A
authors.lastname: Varan
authors.forename: Abduselam
authors.initials: A
authors.lastname: Batu
authors.forename: Ayşenur
authors.initials: A
authors.lastname: Cila
authors.forename: Figen
authors.initials: F
authors.lastname: Soylemezoğlu
authors.forename: Serdar
authors.initials: S
authors.lastname: Balcı
authors.forename: Nejat
authors.initials: N
authors.lastname: Akalan
authors.forename: Faruk
authors.initials: F
authors.lastname: Zorlu
authors.forename: Canan
authors.initials: C
authors.lastname: Akyüz
authors.forename: Tezer
authors.initials: T
authors.lastname: Kutluk
authors.forename: Münevver
authors.initials: M
authors.lastname: Büyükpamukçu
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D020339:Optic Nerve Glioma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011239:Prednisolone / Q000008:administration & dosage; D011344:Procarbazine / Q000008:administration & dosage; D011379:Prognosis; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D011344:Procarbazine; D014750:Vincristine; D005047:Etoposide; D003520:Cyclophosphamide; D011239:Prednisolone; D002945:Cisplatin