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  • Updated 04.07.2022
  • Released 03.05.2002
  • Expires For CME 04.07.2025

Central neurocytoma

Introduction

Overview

Central neurocytomas are typically low-grade intracranial lesions comprising approximately 0.5% of all brain tumors (26; 27; 47). These tumors are classified by the World Health Organization as grade II, with a high rate of recurrence (39; 47; 27). Although central neurocytomas commonly manifest within the cerebral ventricular system (21; 22; 42), they have also been reported in the cerebral hemispheres, limbic system, spinal cord, and other areas of the central nervous system (34; 38; 09; 49; 55; 27; 42; 54).

Central neurocytomas are similar in radiological and histological presentation to other brain tumors, such as glioblastomas, oligodendrogliomas, astrocytomas, pleomorphic xanthoastrocytomas, and ependymomas; therefore, the diagnosis requires a careful evaluation (27; 54). Diagnosis is generally achieved by a combination of MRI or CT imaging, with immunohistochemical confirmation of specific molecular markers (27). More advanced techniques such as next-generation sequencing and DNA methylation profiling can play an additional role in confirming the diagnosis. Prognosis is generally favorable, and primary treatments may include surgical resection or stereotactic radiosurgery (07; 32; 39; 27).

Key points

• Central neurocytomas are rare, brain tumors located within the ventricular system.

• Obstruction of the ventricles by central neurocytomas can lead to hydrocephalus, intracranial hypertension, and mass effect.

• The presence of the immunohistochemical marker synaptophysin can aid in the differential diagnosis of central neurocytomas.


• Maximal safe resection with adjuvant radiotherapy is most frequently utilized for treating central neurocytomas and has a favorable prognosis.


• Stereotactic radiosurgery is an alternative primary treatment with a promising prognosis.


• These tumors have high rates of recurrence and may occur after long latent periods.

Historical note and terminology

Central neurocytomas are thought to be derived from neuronal cells, neuronal progenitor cells, neuronal stem cells, or multipotent precursor cells that arise from the fornix, wall of the lateral ventricles, or the septum pellucidum (57; 19; 26; 27; 51; 07; 56; 21; 32; 47). Despite multiple sources of support for these hypotheses, the known incidence of occurrences throughout the rest of the central nervous system has yet to be elucidated (34; 38; 09; 49; 55; 27; 42; 54).

“Central” neurocytoma is used to reference those lesions that reside within the cerebral ventricular system, whereas “extraventricular,” and occasionally “atypical,” neurocytomas reside elsewhere in the central nervous system.

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