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

作者：Miwa Tomoru^*; Oi Shizuo; Nonaka Yuichiro; Tamogami Ryo; Sasaki Hikaru; Yoshinari Satoshi; Ida Hiroyuki

来源：Child's Nervous System, 2011, 27(8): 1341-1346.

DOI：10.1007/s00381-011-1450-9

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

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.
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.
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.
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.

• 出版日期2011-8