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Erschienen in: Brain Tumor Pathology 4/2017

22.08.2017 | Case Report

Encouraging option of multi-staged gross total resection for a C11orf-RelA fusion-positive supratentorial anaplastic ependymoma

verfasst von: Taishi Nakamura, Kohei Fukuoka, Junji Ikeda, Masahiro Yoshitomi, Naoko Udaka, Reo Tanoshima, Kensuke Tateishi, Shoji Yamanaka, Koichi Ichimura, Tetsuya Yamamoto

Erschienen in: Brain Tumor Pathology | Ausgabe 4/2017

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Abstract

Ependymomas are primary neuroepithelial malignancies that mainly occur during childhood, and arise from ependymal cells along the ventricular systems of the CNS. Recently, it was elucidated that two-thirds of supratentorial (ST) ependymomas harbor oncogenic fusions of RELA, whose protein product is the principal effector of canonical NF-κB signaling. RELA fusion proteins activate signaling for tumor proliferation and malignant progression, resulting in poorer prognoses in these patients compared to those in patients with other ST ependymomas. In this study, we encountered a case of C11orfRelA fusion-positive ST anaplastic ependymoma that was diagnosed in first tumor resection surgery of multi-staged gross total resection with molecular evidence. In ependymomas, regardless of tumor location or pathological grade, subtotal resection is associated with higher rates of mortality compared with GTR. Molecular analysis based on the application of recent molecular knowledge for ST ependymomas performs a role in appropriate and individualized treatment strategies.
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Metadaten
Titel
Encouraging option of multi-staged gross total resection for a C11orf-RelA fusion-positive supratentorial anaplastic ependymoma
verfasst von
Taishi Nakamura
Kohei Fukuoka
Junji Ikeda
Masahiro Yoshitomi
Naoko Udaka
Reo Tanoshima
Kensuke Tateishi
Shoji Yamanaka
Koichi Ichimura
Tetsuya Yamamoto
Publikationsdatum
22.08.2017
Verlag
Springer Singapore
Erschienen in
Brain Tumor Pathology / Ausgabe 4/2017
Print ISSN: 1433-7398
Elektronische ISSN: 1861-387X
DOI
https://doi.org/10.1007/s10014-017-0297-5

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