title: Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases.
abstract: OBJECTIVE
  Gliomatosis cerebri (GC) is a rare neoplasm including a variety of tumors, with extremely variable evolution and heterogeneity of prognosis. It may appear either de novo or after a focal glioma, involve predominantly the white or the gray matter, and concern either pediatric or adult patients. We focused on primary GC involving exclusively gray matter in a pediatric population in order better to define the presentation and outcome of this disease.
  
  
  METHODS
  We reviewed the databases of seven Departments of Pediatric Oncology to identify pediatric cases of GC between 1990 and 2007. Patients were included if they demonstrated a diffuse infiltrative process involving gray matter in magnetic resonance imaging (MRI) and histological tissue analyses, confirming a proliferative glial disorder.
  
  
  RESULTS
  Fourteen patients with a median age of 8 years were identified. Epilepsy was the main presenting symptom. Brain MRI showed a lesion of the temporal and insular cerebral cortex associated with tumoral infiltration of the thalami and the basal ganglia. Histological examination confirmed the diagnosis of high-grade glioma. Prognosis was always very gloomy in the short term, with a median survival of less than a year.
  
  
  CONCLUSIONS
  This rare entity, whose prognosis is appalling whatever the treatment proposed, should be clearly identified within the heterogeneous group of GC in the same way as diffuse intrinsic pontine gliomas have been identified among brain stem tumors. Systematic biopsies appear essential to permit the molecular studies which will assist in guiding the choice of future targeted treatments.
authors.affiliation: Department of Pediatric Oncology, Pontchaillou University Hospital, Rennes, France.
authors.forename: Céline
authors.initials: C
authors.lastname: Chappé
authors.forename: Laurent
authors.initials: L
authors.lastname: Riffaud
authors.forename: Catherine
authors.initials: C
authors.lastname: Tréguier
authors.forename: Béatrice
authors.initials: B
authors.lastname: Carsin-Nicol
authors.forename: David
authors.initials: D
authors.lastname: Veillard
authors.forename: Dan Cristian
authors.initials: DC
authors.lastname: Chiforeanu
authors.forename: Jacques
authors.initials: J
authors.lastname: Grill
authors.forename: Didier
authors.initials: D
authors.lastname: Frappaz
authors.forename: Nicolas
authors.initials: N
authors.lastname: André
authors.forename: Fréderic
authors.initials: F
authors.lastname: Millot
authors.forename: Matthieu
authors.initials: M
authors.lastname: Vinchon
authors.forename: Nicolas
authors.initials: N
authors.lastname: Sirvent
authors.forename: Christine
authors.initials: C
authors.lastname: Edan
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000145:classification / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018302:Neoplasms, Neuroepithelial / Q000145:classification / Q000473:pathology*; D036421:Nerve Fibers, Unmyelinated / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies