Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state
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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.
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.
- Kieran MW, Walker D, Frappaz D, Prados M (2010) Brain tumors: from childhood through adolescence into adulthood. J Clin Oncol 28:4783–4789 CrossRef
- Teasdale G, Jennett B (1976) Assessment and prognosis of coma after head injury. Acta Neurochir (Wien) 34:45–55 CrossRef
- Hirose Y, Aldape K, Takahashi M, Berger MS, Feuerstein BG (2001) Tissue microdissection and degenerate oligonucleotide primed-polymerase chain reaction (DOP-PCR) is an effective method to analyze genetic aberrations in invasive tumors. J Mol Diagn 3:62–67 CrossRef
- Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N et al (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352:978–986 CrossRef
- Brown HG, Kepner JL, Perlman EJ, Friedman HS, Strother DR, Duffner PK et al (2000) "Large cell/anaplastic" medulloblastomas: a Pediatric Oncology Group Study. J Neuropathol Exp Neurol 59:857–865
- Giangaspero F, Rigobello L, Badiali M, Loda M, Andreini L, Basso G et al (1992) Large-cell medulloblastomas. A distinct variant with highly aggressive behavior. Am J Surg Pathol 16:687–693 CrossRef
- Giangaspero F, Eberhart CG, Haapasalo H, Pietsch T, Wiestler OD, Ellison DW (2007) Medullobalstoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th edn. IARC Press, Lyon, pp 132–140
- Colak A, Belen D, Onol B, Saglam S (1993) Adult medulloblastoma presenting as a large neck tumor. Case report. J Neurosurg Sci 37:171–173
- McComb JG, Davis RL, Isaacs H Jr, Landing BH (1980) Medulloblastoma presenting as neck tumors in 2 infants. Ann Neurol 7:113–117 CrossRef
- Batra SK, McLendon RE, Koo JS, Castelino-Prabhu S, Fuchs HE, Krischer JP et al (1995) Prognostic implications of chromosome 17p deletions in human medulloblastomas. J Neurooncol 24:39–45 CrossRef
- Pfister S, Remke M, Benner A, Mendrzyk F, Toedt G, Felsberg J et al (2009) Outcome prediction in pediatric medulloblastoma based on DNA copy-number aberrations of chromosomes 6q and 17q and the MYC and MYCN loci. J Clin Oncol 27:1627–1636 CrossRef
- Lo KC, Ma C, Bundy BN, Pomeroy SL, Eberhart CG, Cowell JK (2007) Gain of 1q is a potential univariate negative prognostic marker for survival in medulloblastoma. Clin Cancer Res 13:7022–7028 CrossRef
- von Hoff K, Hartmann W, von Bueren AO, Gerber NU, Grotzer MA, Pietsch T et al (2010) Large cell/anaplastic medulloblastoma: outcome according to myc status, histopathological, and clinical risk factors. Pediatr Blood Cancer 54:369–376 CrossRef
- Eberhart CG, Kratz JE, Schuster A, Goldthwaite P, Cohen KJ, Perlman EJ et al (2002) Comparative genomic hybridization detects an increased number of chromosomal alterations in large cell/anaplastic medulloblastomas. Brain Pathol 12:36–44 CrossRef
- Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state
Child's Nervous System
Volume 27, Issue 8 , pp 1341-1346
- Cover Date
- Print ISSN
- Online ISSN
- Additional Links
- Brain death
- Comparative genomic hybridization
- Extracranial extension
- Large cell medulloblastoma
- Industry Sectors
- Author Affiliations
- 1. 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
- 2. Department of Neurosurgery, Keio University School of Medicine, Tokyo, Japan
- 3. Department of Pediatrics, Jikei University School of Medicine, Tokyo, Japan