title: Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 trial.
abstract: BACKGROUND
  We describe demographic data of disseminated childhood low-grade glioma (DLGG) prospectively recruited in the HIT-LGG 1996 study and evaluate the impact of primary chemotherapy (CT) on the outcome of these tumors, which have previously only been described in small and retrospective series.
  
  
  METHODS
  The multicenter study HIT-LGG 1996 accrued 1181 children and adolescents with low-grade glioma. 61 patients (5.2%) had tumor dissemination, with 2.8% being present at diagnosis. Frequencies of dissemination for different subgroups were calculated. Efficiency of first-line CT with vincristine/carboplatin was defined in 24 children with dissemination prior to first-line non-surgical-treatment.
  
  
  RESULTS
  Incidence of dissemination was high among infants (16%) with hypothalamic-chiasmatic-glioma (HCG) and diencephalic syndrome. A relevant percentage of HCG showed isolated spinal dissemination. CT achieved objective and overall response rates of 25% and 79% of the primary tumor and a similar response of disseminated lesions. Clinical stabilization or improvement could be achieved in the majority of patients during treatment. However, 20 of 24 patients experienced further progression and 5-year PFS was 6%. Dissemination prior to CT was a negative prognostic factor for PFS within the study (P = 0.005). Overall-survival of primary DLGG was inferior compared to LGG without dissemination at diagnosis (P < 0.001).
  
  
  CONCLUSIONS
  Complete MRI scan should be a standard diagnostic procedure in young children with hypothalamic-chiasmatic tumors especially if presenting with diencephalic syndrome. Dissemination in childhood LGG relates to impaired PFS. CT delays progression for responders. Multicenter studies have to evaluate the efficacy of extended treatment strategies in DLGG to improve outcome.
authors.affiliation: Children's Hospital of Augsburg, D-86156 Augsburg, Germany. stephan.v.hornstein@klinikum-augsburg.de
authors.forename: Stephan
authors.initials: S
authors.lastname: von Hornstein
authors.forename: Rolf-D
authors.initials: RD
authors.lastname: Kortmann
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
authors.forename: Angela
authors.initials: A
authors.lastname: Emser
authors.forename: Monika
authors.initials: M
authors.lastname: Warmuth-Metz
authors.forename: Niels
authors.initials: N
authors.lastname: Soerensen
authors.forename: Ronald
authors.initials: R
authors.lastname: Straeter
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Barbara
authors.initials: B
authors.lastname: Thieme
authors.forename: Astrid K
authors.initials: AK
authors.lastname: Gnekow
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000556:secondary / Q000628:therapy*; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D011878:Radiotherapy; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D016190:Carboplatin