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Pediatric glioblastoma with oligodendroglioma component: aggressive clinical phenotype with distinct molecular characteristics.

Abstract
The 2007 World Health Organization classification defined a new variant of glioblastoma (GBM) containing oligodendroglioma foci as GBM with an oligodendroglioma component (GBMO), which shows a favorable clinical outcome compared with "classic" GBM. However, all of the reported cases of GBMO have been adult cases, with no previous reports of pediatric cases. In this report, we demonstrated molecular characteristics of a pediatric GBMO case, showing aggressive clinical behavior with 8-month overall survival. The case showed neither isocitrate dehydrogenase 1/2 genes (IDH1/2) mutation nor 1p/19q co-deletion, a hallmark of oligodendroglioal tumors. In addition, microsatellite instability, leading to the putative mechanism of temozolomide (TMZ) resistance, was frequently detected. Molecular genetic analysis may provide critical prognostic and therapeutic insights, especially for the pediatric glioma containing oligodendroglioma components.
AuthorsMasahiro Mizoguchi, Nobuhiro Hata, Satoshi O Suzuki, Yutaka Fujioka, Hideki Murata, Toshiyuki Amano, Akira Nakamizo, Koji Yoshimoto, Toru Iwaki, Tomio Sasaki
JournalNeuropathology : official journal of the Japanese Society of Neuropathology (Neuropathology) Vol. 33 Issue 6 Pg. 652-7 (Dec 2013) ISSN: 1440-1789 [Electronic] Australia
PMID23530875 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Copyright© 2013 Japanese Society of Neuropathology.
Chemical References
  • Dacarbazine
  • Temozolomide
Topics
  • Brain Neoplasms (genetics, pathology, therapy)
  • Chemoradiotherapy
  • Child
  • Dacarbazine (analogs & derivatives, therapeutic use)
  • Fatal Outcome
  • Glioblastoma (genetics, pathology, therapy)
  • Humans
  • Male
  • Microsatellite Instability
  • Neoplasms, Complex and Mixed (genetics, pathology, therapy)
  • Neurosurgical Procedures
  • Oligodendroglioma (genetics, pathology, therapy)
  • Phenotype
  • Temozolomide

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