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A case of anaplastic clear-cell ependymoma presenting with high erythropoietin concentration and 1p/19q deletions

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Abstract

We describe a 19-year-old woman with onset of epileptic seizure, and a small mural nodule and multicystic lesions with severe brain edema located in the right frontal lobe. At surgery, the tumor and a clear margin was removed, and symptoms improved postoperatively. Extended local radiotherapy (60 Gy) was performed. Histopathological examination revealed oligodendroglioma-like tumor cells with a perinuclear halo. The tumor cells extended processes toward CD34-positive proliferating vessels, which resemble a basement membrane. These proliferating vessels formed a tumor membrane so that there was a clear margin between the tumor and brain tissue. Tumor cells were positive for epithelial membrane antigen in a dot-like pattern. MIB-1 staining index was 50.6%. Electron microscopy showed cilia and zipper-like junctions, and anaplastic clear-cell ependymoma grade III was diagnosed. A characteristic of the case was formation of a tumor membrane by proliferating tumor blood vessels. Fluorescence in situ hybridization showed 1p/19q deletions, and the concentration of erythropoietin in the cyst fluid was abnormally high, at 1,859.4 mIU/ml. Erythropoietin and erythropoietin receptors were verified with immunohistochemical staining.

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Acknowledgments

The authors express their deep gratitude to Professor Osami Kubo, Department of Neurosurgery, Tokyo Women’s University, and Dr. Takashi Komori, Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, for their valuable discussion and advice leading to completion of this manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, Hirosaki University Graduate School of Medicine, Zaifu-cho 5, Hirosaki, Aomori, 036-8562, Japan

    Kenichiro Asano, Kanae Kudo & Hiroki Ohkuma

  2. Department of Neuropathology, Institute of Brain Science, Hirosaki University Graduate School of Medicine, Hirosaki, Japan

    Fumiaki Mori & Koichi Wakabayashi

Authors
  1. Kenichiro Asano
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  2. Kanae Kudo
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  3. Fumiaki Mori
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  4. Hiroki Ohkuma
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  5. Koichi Wakabayashi
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Correspondence to Kenichiro Asano.

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Asano, K., Kudo, K., Mori, F. et al. A case of anaplastic clear-cell ependymoma presenting with high erythropoietin concentration and 1p/19q deletions. Brain Tumor Pathol 28, 317–327 (2011). https://doi.org/10.1007/s10014-011-0043-3

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  • DOI: https://doi.org/10.1007/s10014-011-0043-3

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