Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state

Tomoru Miwa, Shizuo Oi, Yuichiro Nonaka, Ryo Tamogami, Hikaru Sasaki, Satoshi Yoshinari, Hiroyuki Ida

Research output: Contribution to journalArticle

Abstract

Introduction: 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. Materials and 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.

Original languageEnglish
Pages (from-to)1341-1346
Number of pages6
JournalChild's Nervous System
Volume27
Issue number8
DOIs
Publication statusPublished - 2011 Aug 1

Keywords

  • Brain death
  • Comparative genomic hybridization
  • Extracranial extension
  • Hydrocephalus
  • Large cell medulloblastoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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