Intraventricular neurocytomas

Central neurocytomas (CNCs) are World Health Organization 11 benign central nervous system (CNS) neoplasms first described in 1982 by Hassoun and his colleagues. Hallmark features of CNC include (1) occurrence in the lateral ventricle of young adults, (2) a well-circumscribed isodense to hyperdense mass with contrast enhancement on CT and isointense to hyperintense compared with normal brain parenchyma on T1- and T2-weighted MRI, (3) resemblance to oligodendroglioma on light microscopy, (4) neuronal origin seen in electron microscopy and immunohistochemistry, and (5) favorable prognosis with benign biologic behavior. CNCs comprise 0.1% to 0.5% of all CNS neoplasms based on pathologic review at several neurosurgery centers. A population-based incidence has not been established, in part because of the paucity of cases. Given its recent distinction as a unique tumor and its low incidence, most reports of CNC are from the pathologic literature with little data regarding its management. Furthermore, many early cases of CNC were misdiagnosed, and treatment was based on the presumed diagnosis of oligodendroglioma or ependymoma. Accordingly, this article presents a comprehensive review of the literature and proposes a management paradigm for the treatment of CNC.