Central neurocytomas (CN) are uncommon
tumors of the central nervous system, most descriptions of which available in the literature are in the form of isolated case reports and small series. Owing to this rare incidence, diagnosis and management of this
neoplasm remain controversial. Usually, these
tumors affect lateral ventricles of young adults and display characteristic neuroimaging and histomorphologic findings.
Neurocytomas often mimic
oligodendrogliomas when confirmation of diagnosis rests on immunohistochemistry, ultrastructure, and genetic studies. Extraventricular
neurocytomas, situated entirely within the brain parenchyma and spinal cord, have also been reported. Typically, CN are associated with a favorable outcome although cases with more aggressive
clinical course with recurrences are not unknown. MIB-1 labeling index (LI) of >2% often heralds poor prognosis and tumour recurrence. Safe maximal resection is presently considered the ideal therapeutic option, with best long-term prognosis in terms of local control and survival. The role of
adjuvant radiotherapy apparently seems to benefit patients with incomplete resection and in atypical
neurocytoma. Utility of other therapeutic regimen, however, remains shrouded in controversy. Epidemiology, histogenesis, clinical profile, histology, neuroimaging and therapeutic modalities of
neurocytomas have been comprehensively reviewed, with special emphasis on CN and extraventricular
neurocytomas and their atypical counterparts.