Central neurocytoma

Isaac Yang MD (

Dr. Yang of UCLA Neurosurgery received honorariums from Baxter, Brainlab, and GS Medical and a research grant from Stryker.

)
Rimas V Lukas MD, editor. (

Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from AbbVie and Novocure for speaking engagements, from Eisai for consulting work, and from Monetris as an advisory board member.

)
Originally released March 5, 2002; last updated August 29, 2019; expires August 29, 2022

This article contains a clinical history of central neurocytomas, including clinical manifestations, prognosis and complications, histological and genetic makeup, treatment, and differential diagnoses. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Central neurocytomas are benign intracranial tumors composing up to 0.5% of all brain tumors (Sander et al 2019). They are classified by the World Health Organization as grade II (Nakamura et al 2019; Sander et al 2019). Although central neurocytoma commonly manifest within the cerebral ventricular system (Kane et al 2011; Kawano et al 2015), they have also been reported in the cerebral hemispheres, limbic system, spinal cord, cerebellum, brainstem, and retina (Louis et al 1990; Metcalf et al 1993; Coca et al 1994; Sgouros et al 1994; Tatter et al 1994).

Central neurocytomas are similar in radiological and histological presentation to other brain tumors, such as oligodendrogliomas, astrocytomas, and ependymomas, and, therefore, require careful differential diagnosis. Diagnosis is generally achieved by a combination of MRI or CT imaging with immunohistochemical confirmation of specific molecular markers. Prognosis is generally good, and primary treatments may include surgical resection or stereotactic radiosurgery (Chen et al 2017; Lee et al 2018; Nakamura et al 2019).

Key points

 

• Central neurocytomas are rare, brain tumors located in 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 good prognosis.

 

• Stereotactic radiosurgery is becoming an alternative primary treatment for central neurocytoma.

 

• These tumors are at risk for recurrence, and recurrences may occur after prolonged intervals.

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 (Tsuchida et al 1996; Ishiuchi and Tamura 1997; Sim et al 2006; Taupin 2008; Kane et al 2011; Chen et al 2012; Kawano et al 2015; Lee et al 2016; Sander et al 2019).

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

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