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A clinicopathologic study of extraventricular neurocytoma

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Abstract

In 2007, extraventricular neurocytoma was classified as a separate entity among glioneuronal tumors. However, extraventricular neurocytoma is not fully understood and may be misdiagnosed. Here, we describe the clinical and pathological features, prognoses, and treatments of 13 extraventricular neurocytoma cases, and compare their immunophenotypes with those of oligodendroglioma, diffuse astrocytoma, and ependymoma. Six typical and 7 atypical cases comprised the 13 extraventricular neurocytoma cases. Histological features included oligodendroglioma-like perinuclear halo, neuropil-like matrix, ganglion or ganglioid cells, perivascular pseudorosettes, vessel hyalinization, calcifications, and myxoid degeneration. Atypical histological features included increased mitotic figures, focal necrosis, endothelial cell proliferation, and/or a Ki-67 index of >2%. All lesions expressed synaptophysin and microtubule-associated protein-2, which distinguished them from other similar tumors. Two patients with atypical extraventricular neurocytoma had tumor recurrence, one of whom had cerebrospinal fluid dissemination, suggesting that atypical histological features might represent adverse prognostic factors. In conclusion, the present study identified morphological and immunohistochemical features that would aid the differential diagnosis of extraventricular neurocytoma. In addition, radiotherapy with subtotal resection could be considered an effective treatment for extraventricular neurocytoma, but because a pediatric patient died of intracranial hemorrhage during radiotherapy, radiotherapy-related side effects should be considered, especially when treating children. Additional cases with long-term follow-up are needed to develop optimal management protocols for extraventricular neurocytoma.

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Funding

This study was funded by Grants 2013BAI09B03 (National Key Technology Research and Development Program of the Ministry of Science and Technology of China) and BIBD-PXM2013_014226_07_000084 (Beijing Institute for Brain Disorders).

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    Authors and Affiliations

    1. Department of Neuropathology, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, China National Clinical Research Center for Neurological Diseases (NCRC-ND), Center of Brain Tumor, Beijing Institute for Brain Disorders, Beijing Key Laboratory of Brain Tumor, 6 Tiantan Xili, Dongcheng District, Beijing, 100050, China

      Li Xu, Junmei Wang, Zhaoxia Liu, Jingyi Fang, Jiang Du, Yanjiao He & Guilin Li

    2. Institute of Medical Information, Chinese Academy of Medical Sciences, Beijing, China

      Zhaolian Ouyang

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    1. Li Xu
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    2. Zhaolian Ouyang
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    Correspondence to Guilin Li.

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    The authors declare that they have no conflict of interest.

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    All procedures were performed in accordance with the ethical standards of the responsible committee on human experimentation of Capital Medical University, Beijing, China, and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

    Additional information

    Li Xu and Zhaolian Ouyang have contributed equally to this work.

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    Xu, L., Ouyang, Z., Wang, J. et al. A clinicopathologic study of extraventricular neurocytoma. J Neurooncol 132, 75–82 (2017). https://doi.org/10.1007/s11060-016-2336-1

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    • DOI: https://doi.org/10.1007/s11060-016-2336-1

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