title: Long-term outcome of centrally located low-grade glioma in children.
abstract: BACKGROUND
  Optimal management of children with centrally located low-grade glioma (LGG) is unclear. Initial interventions in most children are chemotherapy in younger and radiation therapy (RT) in older children. A better understanding of the inherent risk factors along with the effects of interventions on long-term outcome can lead to reassessment of the current approaches to minimize long-term morbidity.
  
  
  METHODS
  To reassess the current treatment strategies of centrally located LGG, we compared the long-term survival and morbidity of different treatment regimens. Medical records of patients primarily treated at Texas Children's Cancer and Hematology Centers between 1987 and 2008 were reviewed.
  
  
  RESULTS
  Forty-seven patients with a median follow-up of 79 months were included in the analysis. The 5-year overall survival and progression-free survival (PFS) for all patients were 96% and 53%, respectively. The 5-year PFS for those treated initially with RT (12 patients; median age, 11 years [range, 3-15 years]) and with chemotherapy (28 patients; median age, 2 years [range 0-8 years]) were 76% and 37%, respectively (log-rank test P = .02). Among children who progressed after chemotherapy, the 5-year PFS after salvage RT was 55%. Patients diagnosed at a younger age (<5 years) were more likely to experience endocrine abnormalities (Fisher exact test; P<.00001).
  
  
  CONCLUSIONS
  Effective and durable tumor control was obtained with RT as initial treatment. In younger patients, chemotherapy can delay the use of RT; however, frequent progression and long-term morbidity are common. More effective and less toxic therapies are required in these patients, the majority of whom are long-term survivors.
authors.affiliation: Texas Children's Cancer and Hematology Centers, Houston, Texas, USA.
authors.forename: Keita
authors.initials: K
authors.lastname: Terashima
authors.forename: Kevin
authors.initials: K
authors.lastname: Chow
authors.forename: Jeremy
authors.initials: J
authors.lastname: Jones
authors.forename: Charlotte
authors.initials: C
authors.lastname: Ahern
authors.forename: Eunji
authors.initials: E
authors.lastname: Jo
authors.forename: Benjamin
authors.initials: B
authors.lastname: Ellezam
authors.forename: Arnold C
authors.initials: AC
authors.lastname: Paulino
authors.forename: M Fatih
authors.initials: MF
authors.lastname: Okcu
authors.forename: Jack
authors.initials: J
authors.lastname: Su
authors.forename: Adekunle
authors.initials: A
authors.lastname: Adesina
authors.forename: Anita
authors.initials: A
authors.lastname: Mahajan
authors.forename: Robert
authors.initials: R
authors.lastname: Dauser
authors.forename: William
authors.initials: W
authors.lastname: Whitehead
authors.forename: Ching
authors.initials: C
authors.lastname: Lau
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
mesh_terms: D000293:Adolescent; D000342:Affective Symptoms / Q000453:epidemiology / Q000209:etiology; D001254:Astrocytoma / Q000473:pathology / Q000628:therapy; D014408:Biomarkers, Tumor / Q000032:analysis*; D001932:Brain Neoplasms / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000453:epidemiology / Q000209:etiology; D018572:Disease-Free Survival; D004700:Endocrine System Diseases / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005910:Glioma / Q000737:chemistry / Q000188:drug therapy / Q000235:genetics / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy*; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D008499:Medical Records; D060787:Neoplasm Grading; D009422:Nervous System Diseases / Q000453:epidemiology / Q000209:etiology; D015514:Oncogene Proteins, Fusion / Q000032:analysis*; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D017741:Survivors / Q000706:statistics & numerical data; D013781:Texas / Q000453:epidemiology; D016896:Treatment Outcome; D014786:Vision Disorders / Q000453:epidemiology / Q000209:etiology
chemical_list: C568616:BRAF-KIAA1549 fusion protein, human; D014408:Biomarkers, Tumor; D015514:Oncogene Proteins, Fusion
keywords: BRAF-KIAA1549 fusion protein; chemotherapy; childhood cerebral astrocytoma; childhood optic nerve glioma; hypothalamic-chiasmatic neoplasms; long-term effects; pilocytic astrocytoma; radiotherapy