title: An unusual form of superficially disseminated glioma in children: report of 3 cases.
abstract: Three children, aged 4, 5, and 9 years, had an insidious onset of ataxia. Magnetic resonance imaging (MRI) showed hydrocephalus and countless foci of high T2 signal coating the cerebellum, basilar cisterns, brainstem, and fourth ventricle. Similar lesions were present in the spinal cord. Symptoms were relatively mild given the massive tumor burden. Biopsies were composed of superficially infiltrating cells with oligodendroglioma-like features (perinuclear halos and cytologic monotony) and microcysts. Classical cytogenetic analysis of 2 cases showed normal karyotypes. Chromosome fluorescence in situ hybridization revealed 1p36 deletion with intact 19q in 2 cases and no abnormality in one. A similar combination of clinical, MRI, and histopathologic findings has been reported previously in 10 other cases. The pathologic findings suggest a glioma with diffuse or multifocal superficial origin and do not correspond to a described entity in the current World Health Organization (WHO) classification of brain tumors.
authors.affiliation: Department of Pathology, Akron Children's Hospital and Northeastern Ohio Universities College of Medicine, Akron, OH 44308, USA. dagamanolis@chmca.org
authors.forename: Dimitri P
authors.initials: DP
authors.lastname: Agamanolis
authors.forename: Christos D
authors.initials: CD
authors.lastname: Katsetos
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Klonk
authors.forename: Henry M
authors.initials: HM
authors.lastname: Bartkowski
authors.forename: Srinivas
authors.initials: S
authors.lastname: Ganapathy
authors.forename: Susan M
authors.initials: SM
authors.lastname: Staugaitis
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Kuerbitz
authors.forename: Donna F
authors.initials: DF
authors.lastname: Patton
authors.forename: Ali
authors.initials: A
authors.lastname: Talaizadeh
authors.forename: Bruce H
authors.initials: BH
authors.lastname: Cohen
mesh_terms: D001706:Biopsy; D001921:Brain / Q000378:metabolism / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D002872:Chromosome Deletion; D002878:Chromosomes, Human, Pair 1; D018450:Disease Progression; D005260:Female; D005910:Glioma / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D006801:Humans; D006849:Hydrocephalus; D019394:Ki-67 Antigen / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008297:Male; D046529:Microscopy, Electron, Transmission; D019581:Neuropil / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D009418:S100 Proteins / Q000378:metabolism
chemical_list: D019394:Ki-67 Antigen; D009418:S100 Proteins