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.
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Acknowledgments
We thank Mrs. Kiyomi Koide and Mrs. Naoko Tsuzaki of the Department of Neurosurgery, Keio University, for their technical assistance.
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Miwa, T., Oi, S., Nonaka, Y. et al. Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state. Childs Nerv Syst 27, 1341–1346 (2011). https://doi.org/10.1007/s00381-011-1450-9
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DOI: https://doi.org/10.1007/s00381-011-1450-9