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

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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 C11orf–RelA 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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Acknowledgements

This work is dedicated to the memory of Professor Nobutaka Kawahara.

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Authors and Affiliations

  1. Department of Neurosurgery, Graduate School of Medicine, Yokohama City University, Yokohama, Japan

    Taishi Nakamura, Kensuke Tateishi & Tetsuya Yamamoto

  2. Division of Brain Tumor Translational Research, National Cancer Center Research Institute, Tokyo, Japan

    Kohei Fukuoka & Koichi Ichimura

  3. Department of Pediatrics, Graduate School of Medicine, Yokohama City University, Yokohama, Japan

    Junji Ikeda, Masahiro Yoshitomi & Reo Tanoshima

  4. Department of Pathology, Graduate School of Medicine, Yokohama City University, Yokohama, Japan

    Naoko Udaka & Shoji Yamanaka

Authors
  1. Taishi Nakamura
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  2. Kohei Fukuoka
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  3. Junji Ikeda
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  4. Masahiro Yoshitomi
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  5. Naoko Udaka
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  6. Reo Tanoshima
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  7. Kensuke Tateishi
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  8. Shoji Yamanaka
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  9. Koichi Ichimura
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  10. Tetsuya Yamamoto
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Correspondence to Taishi Nakamura.

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Nakamura, T., Fukuoka, K., Ikeda, J. et al. Encouraging option of multi-staged gross total resection for a C11orf-RelA fusion-positive supratentorial anaplastic ependymoma. Brain Tumor Pathol 34, 160–164 (2017). https://doi.org/10.1007/s10014-017-0297-5

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  • DOI: https://doi.org/10.1007/s10014-017-0297-5

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