title: Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state.
abstract: BACKGROUND
  The authors describe the case of a 29-month-old boy who presented with acute non-communicating hydrocephalus caused by a small tumor in the fourth ventricle. He became brain-dead immediately and remained stable in that condition.
  
  
  METHODS
  Six months later, despite being in a brain-dead state, a rapid direct tumor extension from the intracranial to extracranial region was observed, and chemoradiotherapy was performed following tumor biopsy. The histopathological diagnosis was large cell medulloblastoma. Although treatment was initially effective, the tumor again aggressively invaded the cervical muscles via the spinal canal. Comparative genomic hybridization (metaphase) analysis revealed a pattern of aberrations predictive of a poor prognosis (+1q, ?17p, +17q, and probable amplification of c-myc gene), and he eventually died 11 months after onset.
  
  
  RESULTS
  Direct invasion of medulloblastoma from the intracranial to extracranial region is extremely rare, and, to our knowledge, this is the first report of medulloblastoma exhibiting rapid extension to the extracranial region in brain-dead state.
  
  
  CONCLUSIONS
  For patients with medulloblastomas, careful observation is needed even in brain-dead state. The etiology of this rare condition as well as the genetic characteristics responsible for aggressive tumor behavior are discussed.
authors.affiliation: Division of Pediatric Neurosurgery, Jikei University Hospital, Women's and Children's Medical Center, Minato-ku, Tokyo, 105-8461, Japan. ten-m@withe.ne.jp
authors.forename: Tomoru
authors.initials: T
authors.lastname: Miwa
authors.forename: Shizuo
authors.initials: S
authors.lastname: Oi
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Nonaka
authors.forename: Ryo
authors.initials: R
authors.lastname: Tamogami
authors.forename: Hikaru
authors.initials: H
authors.lastname: Sasaki
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yoshinari
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Ida
mesh_terms: D001926:Brain Death / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology; D002675:Child, Preschool; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology