title: Cerebellar location may predict an unfavourable prognosis in paediatric high-grade glioma.
abstract: BACKGROUND
  High-grade glioma (HGG) of the cerebellum accounts for only 5% of paediatric HGG. Since little is known about these tumours, the present study aimed at their further characterisation.
  
  
  METHODS
  Twenty-nine paediatric patients with centrally reviewed cerebellar HGG were identified from the HIT-GBM/HIT-HGG database. Clinical and epidemiological data were compared with those of 180 paediatric patients with cortical HGG.
  
  
  RESULTS
  Patients with cerebellar tumours were younger (median age of 7.6 vs 11.7 years, P=0.028), but both groups did not differ significantly with regard to gender, tumour predisposing syndromes, secondary HGG, primary metastasis, tumour grading, extent of tumour resection, chemotherapy regimen, or radiotherapy. Except for an increased incidence of anaplastic pilocytic astrocytoma (APA) in the cerebellar subset (20.7% vs 3.3%; P<0.001), histological entities were similarly distributed in both groups. As expected, tumour grading had a prognostic relevance on survival. Compared with cortical HGG, overall survival in the cerebellar location was significantly worse (median overall survival: 0.92 ± 0.02 vs 2.03 ± 0.32 years; P=0.0064), and tumour location in the cerebellum had an independent poor prognostic significance as shown by Cox-regression analysis (P=0.019).
  
  
  CONCLUSIONS
  High-grade glioma represents a group of tumours with an obviously site-specific heterogeneity associated with a worse survival in cerebellar location.
authors.affiliation: Department of Paediatric and Adolescent Medicine, Universitätsmedizin Mannheim, Medical Faculty Mannheim, Heidelberg University, Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany. michael.karremann@umm.de
authors.forename: M
authors.initials: M
authors.lastname: Karremann
authors.forename: U
authors.initials: U
authors.lastname: Rausche
authors.forename: D
authors.initials: D
authors.lastname: Roth
authors.forename: A
authors.initials: A
authors.lastname: Kühn
authors.forename: T
authors.initials: T
authors.lastname: Pietsch
authors.forename: G H
authors.initials: GH
authors.lastname: Gielen
authors.forename: M
authors.initials: M
authors.lastname: Warmuth-Metz
authors.forename: R-D
authors.initials: RD
authors.lastname: Kortmann
authors.forename: R
authors.initials: R
authors.lastname: Straeter
authors.forename: A
authors.initials: A
authors.lastname: Gnekow
authors.forename: J E A
authors.initials: JE
authors.lastname: Wolff
authors.forename: C M
authors.initials: CM
authors.lastname: Kramm
mesh_terms: D000293:Adolescent; D017677:Age Distribution; D001254:Astrocytoma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D016022:Case-Control Studies; D002528:Cerebellar Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D018303:Ganglioglioma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D005909:Glioblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D005910:Glioma / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D060787:Neoplasm Grading; D009837:Oligodendroglioma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D017678:Sex Distribution; D015173:Supratentorial Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology