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Central neurocytoma (CN), first described by Hassoun and colleagues 1 in 1982, is classified by the World Health Organization as grade II. 2 CNs are characterized as rare, neuronal differentiated tumors that account for less than 1% of all primary brain tumors and often affect young adults. 3–6 CNs are typically located in the third ventricle or the anterior portion of the lateral ventricle, but there are rare cases of extraventricular neurocytoma in other locations, including spinal dissemination. Most neurocytomas have a benign clinical course with an excellent prognosis following surgical resection. As such, it is imperative to make an accurate diagnosis of CN to implement the optimal treatment modality to improve morbidity and mortality. Common differential diagnoses include oligodendrogliomas, ependymomas, non-Hodgkin lymphomas, and astrocytomas. 5, 7–14 This article discusses clinical features related to …