title: Management of pediatric intracranial low-grade gliomas: long-term follow-up after radiation therapy.
abstract: BACKGROUND
  The treatment of pediatric intracranial low-grade gliomas (LGG) generally begins with maximal safe resection. Radiation therapy (RT) and chemotherapy are typically reserved for patients with incomplete resection and/or disease progression. We report long-term treatment outcomes and toxicities in a cohort of pediatric patients with LGG after RT.
  
  
  METHODS
  Thirty-four patients <21 years old with intracranial LGG who were treated with RT at the Johns Hopkins Hospital were included in this retrospective analysis. Patients were evaluated for overall survival (OS), progression-free survival (PFS), recurrence patterns, and treatment toxicities using descriptive statistics, Kaplan-Meier curves, and Cox proportional hazard regressions.
  
  
  RESULTS
  The mean age at diagnosis was 7.9 years (range 1.2-18.3 years) and mean age at RT was 9.8 years (range 3.0-28.9 years). The median follow-up time was 9.8 years after radiation (range 0.8-33.3 years). The estimated 10-year OS and PFS after RT were 92 and 74 %, respectively. Twelve patients had disease progression after RT, and all recurrences were local. Two patients died due to disease progression 2.3 and 9.1 years after RT. One patient had malignant transformation of LGG to high-grade glioma. No significant predictors of PFS were identified on uni- or multivariate analysis. Late effects of LGG and treatment seen were endocrine deficiencies in 16 patients, visual problems in 10 patients, hearing loss in 4 patients, special education requirements for 5 patients, and a vascular injury/demyelination secondary to RT in 1 patient.
  
  
  CONCLUSIONS
  Our study suggests that the use of radiation in patients with intracranial LGG results in excellent OS and PFS with acceptable toxicity at long-term follow-up.
authors.affiliation: Department of Radiation Oncology and Molecular Radiation Sciences, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, 401 N. Broadway, Suite 1440, Baltimore, MD, 21287, USA.
authors.forename: Minh-Phuong
authors.initials: MP
authors.lastname: Huynh-Le
authors.affiliation: Department of Radiation Oncology and Molecular Radiation Sciences, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, 401 N. Broadway, Suite 1440, Baltimore, MD, 21287, USA.
authors.forename: Amanda J
authors.initials: AJ
authors.lastname: Walker
authors.affiliation: Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, 21231, USA.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.affiliation: Department of Neurological Surgery, Johns Hopkins Hospital, Baltimore, MD, 21231, USA.
authors.forename: George I
authors.initials: GI
authors.lastname: Jallo
authors.affiliation: Division of Pediatric Oncology, Johns Hopkins Hospital, Baltimore, MD, 21231, USA.
authors.forename: Kenneth J
authors.initials: KJ
authors.lastname: Cohen
authors.affiliation: Department of Radiation Oncology and Molecular Radiation Sciences, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, 401 N. Broadway, Suite 1440, Baltimore, MD, 21287, USA.
authors.forename: Moody D
authors.initials: MD
authors.lastname: Wharam
authors.affiliation: Department of Radiation Oncology and Molecular Radiation Sciences, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Hospital, 401 N. Broadway, Suite 1440, Baltimore, MD, 21287, USA. Sterezak@jhmi.edu.
authors.forename: Stephanie A
authors.initials: SA
authors.lastname: Terezakis
mesh_terms: D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000000981:diagnostic imaging* / Q000532:radiotherapy*; D002648:Child; D002675:Child, Preschool; D019468:Disease Management; D018572:Disease-Free Survival; D005260:Female; D005910:Glioma / Q000175:diagnosis* / Q000532:radiotherapy*; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D016016:Proportional Hazards Models; D055815:Young Adult
keywords: Low-grade gliomas; Pediatrics; Radiation therapy; Surgery