Central neurocytoma

Cheng Hao Jacky Chen (

Mr. Chen of UCLA Neurosurgery has no relevant financial relationships to disclose.

)
Seung J Lee (

Mr. Lee of UCLA Neurosurgery has no relevant financial relationships to disclose.

)
John P Sheppard (

Mr. Sheppard of UCLA Neurosurgery has no relevant financial relationships to disclose.

)
Thien Nguyen (

Mr. Nguyen of UCLA Neurosurgery has no relevant financial relationships to disclose.

)
Courtney Duong (

Ms. Duong of UCLA Neurosurgery has no relevant financial relationships to disclose.

)
Vera Ong (

Ms. Ong of UCLA Neurosurgery has no relevant financial relationships to disclose

)
Isaac Yang MD (

Dr. Yang of UCLA Neurosurgery received consultant fees from Brainlab.

)
Rimas V Lukas MD, editor. (Dr. Lukas of Northwestern University Feinberg School of Medicine received honorariums from AstraZeneca as an advisory board member and AbbVie as a guest speaker and advisory board member.)
Originally released March 5, 2002; last updated February 9, 2018; expires February 9, 2021

This article includes discussion of central neurocytoma, neurocytoma, and intraventricular neurocytoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Central neurocytoma is a rare, benign intracranial tumor of neuroglial origin. It is classified by the World Health Organization as a grade II tumor. Central neurocytomas manifest within the cerebral ventricular system, most commonly in the lateral ventricles near the foramen of Monro, and attach to the septum pellucidum (Kane et al 2011; Kawano et al 2015). However, extraventricular neurocytomas 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 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 preferred treatment involves maximal safe resection with adjuvant radiotherapy.

Key points

 

• Central neurocytomas are rare, often low-grade 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.

 

• Risk of long-term recurrence after surgical resection is around 20%.

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).

The term “central” neurocytoma is specifically given to neurocytomas that reside within the ventricular system. Much more rarely, extraventricular neurocytomas may occur outside of the ventricular system.

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