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Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination

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Abstract

A rosette-forming glioneuronal tumor (RGNT) was encountered in a 16-year-old Chinese girl. She experienced seizures with loss of consciousness for 1 month prior to diagnosis. A brain MRI revealed multifocal masses occupying all of the ventricular system associated with marked hydrocephalus. A biopsy was performed on the right lateral ventricle using a neuroendoscope and the patient was given postoperative radiotherapy. She was followed for 7 months, and there was no radiological or clinical evidence of tumor progression. Histological examination demonstrated two regions characterized by predominant neurocytic rosettes and scant low grade astrocytoma. No histological anaplasia was present. Immunohistological studies showed that the small round tumor cells forming rosettes were strongly positive for Olig2. A chromosome 1p/19q co-deletion was not detected by FISH analysis. While the pathological features of our case closely resembled those reported in the original description, the diffuse intraventricular growth pattern of the tumor was different from previous examples. Further long-term follow-up studies are required to assess the biological behavior and clinical outcome of the RGNT entity.

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Acknowledgments

The authors thank Ms. Jing-Jing Zhu, Mr. Ming-Shi Gao and Chao Li for technical assistance.

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Authors and Affiliations

  1. Department of Neuropathology, Institute of Neurology, Huashan Hospital, Fudan University, 12 Wu Lu Mu Qi Zhong Road, 200040, Shanghai, China

    Yin Wang, Ji Xiong & Hai-Xia Cheng

  2. Department of Radiology, Huashan Hospital, Fudan University, 200040, Shanghai, China

    Shu-Guang Chu

  3. Department of Pathology, Shanghai Medical College, Fudan University, 200032, Shanghai, China

    Ying Liu

  4. Department of Neurosurgery, Huashan Hospital, Fudan University, 200040, Shanghai, China

    Yong-Fei Wang, Yao Zhao & Ying Mao

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  1. Yin Wang
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  2. Ji Xiong
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  3. Shu-Guang Chu
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  4. Ying Liu
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  5. Hai-Xia Cheng
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  6. Yong-Fei Wang
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Correspondence to Yin Wang.

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Wang, Y., Xiong, J., Chu, SG. et al. Rosette-forming glioneuronal tumor: report of an unusual case with intraventricular dissemination. Acta Neuropathol 118, 813–819 (2009). https://doi.org/10.1007/s00401-009-0569-1

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  • DOI: https://doi.org/10.1007/s00401-009-0569-1

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