Update on the diagnosis, pathogenesis, and treatment strategies for central neurocytoma

• Published in: Journal of clinical neuroscience 2013-09, Vol.20 (9), p.1193-1199
• Main Authors: Patel, Dhruv M, Schmidt, Richard F, Liu, James K
• Format: Article
• Language: English
• Subjects: Animals; Antineoplastic Agents - administration & dosage; Brain Neoplasms - diagnosis; Brain Neoplasms - etiology; Brain Neoplasms - therapy; Brain tumor; Central neurocytoma; Cerebral Ventricle Neoplasms - diagnosis; Cerebral Ventricle Neoplasms - etiology; Cerebral Ventricle Neoplasms - therapy; Humans; Intraventricular tumor; Neurocytoma - diagnosis; Neurocytoma - etiology; Neurocytoma - therapy; Neurology; Radiosurgery - methods; Review article; Treatment Outcome
• ISSN: 0967-5868; 1532-2653
• DOI: 10.1016/j.jocn.2013.01.001

Abstract Central neurocytomas are rare benign tumors of the central nervous system that are typically located in the lateral ventricles. Since they were first reported in the early 1980s, many advancements have been made in terms of their diagnosis and treatment. Despite the progress made, the origin of these rare tumors and effective newer treatment strategies remain elusive. Central neurocytomas represent 0.1–0.5% of all primary brain tumors. Since they are typically intraventricular, these tumors tend to present clinically with hydrocephalus. CT scanning and MRI are useful in localizing these tumors; however, due to their numerous ambiguous features, the ultimate diagnosis relies on immunohistochemistry and electron microscopy studies of sampled tissue. Currently, surgical removal with a gross-total resection of these tumors is the treatment of choice. Various radiotherapy techniques, including both conventional radiotherapy and stereotactic radiosurgery, have been shown to be useful in cases of residual tumor after sub-total resection and tumor recurrence. The benign nature of these tumors tends to offer a favorable outcome for most patients; however, recurrence rates are relatively high and tumors with high-grade features or extraventricular location tend to have a less favorable prognosis. We present a comprehensive review of these rare tumors, including their epidemiology, clinical presentation, radiological presentation, histopathological findings, and options for intervention including surgery, radiation therapy, stereotactic radiosurgery, and chemotherapy.