Pediatric glioblastoma with oligodendroglioma component: Aggressive clinical phenotype with distinct molecular characteristics

作者：Mizoguchi Masahiro^*; Hata Nobuhiro; Suzuki Satoshi O; Fujioka Yutaka; Murata Hideki; Amano Toshiyuki; Nakamizo Akira; Yoshimoto Koji; Iwaki Toru; Sasaki Tomio

来源：Neuropathology, 2013, 33(6): 652-657.

DOI：10.1111/neup.12029

• 1p
• 19q co-deletion
• glioblastoma
• microsatellite instability
• oligodendroglioma
• pediatric
• temozolomide

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.

• 出版日期2013-12