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

研究成果: Article査読

抄録

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.

本文言語English
ページ(範囲)1341-1346
ページ数6
ジャーナルChild's Nervous System
27
8
DOI
出版ステータスPublished - 2011 8月

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康
  • 臨床神経学

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