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Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state.
MedLine Citation:
PMID:  21533576     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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.
Authors:
Tomoru Miwa; Shizuo Oi; Yuichiro Nonaka; Ryo Tamogami; Hikaru Sasaki; Satoshi Yoshinari; Hiroyuki Ida
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-28
Journal Detail:
Title:  Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery     Volume:  -     ISSN:  1433-0350     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-5-2     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8503227     Medline TA:  Childs Nerv Syst     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Division of Pediatric Neurosurgery, Jikei University Hospital, Women's and Children's Medical Center, 3-25-8 Nishi-Shinbashi, Minato-ku, Tokyo, 105-8461, Japan, ten-m@withe.ne.jp.
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