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Neurocytoma: a comprehensive review

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Abstract

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.

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Acknowledgements

The authors acknowledge the contribution of Dr. Ajay Garg, assistant professor, Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, in the form of providing the neuroradiology images.

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Correspondence to Chitra Sarkar.

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William T. Couldwell, Salt Lake City

Since its initial description in 1982 by Hassoun et al., we have learned much about the natural history of this tumor and its response to surgical and radiation therapy. The study of the molecular genetics of this tumor, on the other hand, remains rudimentary, and further work will undoubtedly reap rewards regarding origin, differential diagnosis, and response to treatment. At the present time, the MIB-1 labeling index appears to be the best predictor of proliferative potential and clinical outcome. The role of chemotherapy for treatment of residual tumor has yet to be defined, but promising early reports are emerging. As noted in the report by Sharma et al., larger treatment cohorts need to be evaluated before chemotherapy is incorporated in the conventional treatment schedule.

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Sharma, M.C., Deb, P., Sharma, S. et al. Neurocytoma: a comprehensive review. Neurosurg Rev 29, 270–285 (2006). https://doi.org/10.1007/s10143-006-0030-z

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