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A case of a rosette-forming glioneuronal tumor with clinicopathological features of a dysembryoplastic neuroepithelial tumor and fibroblast growth factor receptor 1 internal tandem duplication

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Abstract

Rosette-forming glioneuronal tumors (RGNTs) are benign WHO grade 1 tumors that occur in the ventricular system, particularly the fourth ventricle. RGNTs and dysembryoplastic neuroepithelial tumors (DNTs) are both categorized as neuronal and mixed neuronal-glial tumors and may be difficult to distinguish. Coexistence of the two tumor types has been reported. Here, we report a pediatric case of RGNT with DNT-like features showing intraventricular dissemination. The tumor occurred in the medial temporal lobe and presented with specific pathological glioneuronal elements including floating neurons, which are typical in DNTs, but was diagnosed as RGNT because of the presence of neurocytic rosettes. Genetic analysis detected fibroblast growth factor receptor 1 internal tandem duplication (FGFR1-ITD) of the tyrosine kinase domain, which was previously reported to be specific for DNT. RGNTs with FGFR1-ITD may show atypical clinical presentation and pathological features.

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Acknowledgements

The authors appreciate Dr. Junji Shibahara, Kyorin University, for his advice on the pathological diagnosis. We thank Emma Longworth-Mills, PhD, from Edanz Group (https://en-author-services.edanz.com/ac) for editing a draft of this manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, Jichi Medical University Saitama Medical Center, 1-847 Amanumacho, Omiya-ku Saitama, Saitama, 330-8503, Japan

    Taku Uchiyama

  2. Department of Pediatric Neurosurgery, Jichi Children’s Medical Center Tochigi, Jichi Medical University, Tochigi, Japan

    Akira Gomi

  3. Department of Human Pathology, Gunma University Graduate School of Medicine, Gunma, Japan

    Sumihito Nobusawa

  4. Department of Pathology, Jichi Medical University, Tochigi, Japan

    Noriyoshi Fukushima & Daisuke Matsubara

  5. Department of Neurosurgery, Jichi Medical University, Tochigi, Japan

    Kensuke Kawai

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  1. Taku Uchiyama
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Correspondence to Taku Uchiyama.

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Uchiyama, T., Gomi, A., Nobusawa, S. et al. A case of a rosette-forming glioneuronal tumor with clinicopathological features of a dysembryoplastic neuroepithelial tumor and fibroblast growth factor receptor 1 internal tandem duplication. Brain Tumor Pathol 38, 250–256 (2021). https://doi.org/10.1007/s10014-021-00397-z

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  • DOI: https://doi.org/10.1007/s10014-021-00397-z

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