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Central Neurocytoma

Last Updated: March 27, 2020

Figure 1: This large central neurocytoma demonstrates its typically cystic feature on axial FLAIR (top row left). This image also shows an entrapped right frontal horn indicated by incomplete suppression of CSF. The SWI image (top row right) shows dark susceptibility artifact that likely represents calcifications commonly seen in these tumors. Central neurocytomas typically have some enhancement, but less than many other tumors, as seen on this postcontrast T1WI (bottom).

Figure 2: Axial T1 post contrast image (top row left) demonstrates a heterogeneously enhancing mass in the left lateral ventricle which appears inseparable from the septum pellucidum. T2-FS image (top row right) demonstrates the cystic and bubbly appearance of this mass. Enhancing components correspond to regions of restricted diffusion and presumed hypercellularity on diffusion weighted (bottom row left) imaging and ADC (bottom row right). Pathology consistent with central neurocytoma.

Figure 3: This right temporal extraventricular neurocytoma mimics the appearance of other low grade primary brain tumors such as ganglioglioma and DNET. It demonstrates T2 hyperintense (top row left – axial, top row right – sagittal, bottom row left – coronal), nonenhancing (bottom row right) cystic components and intermediate signal intensity (top row left, top row right, bottom row left) mildly enhancing (bottom row right) solid component, similar to the more common intraventricular central neurocytoma.

Basic Description

  • Well-marginated, usually benign intraventricular neuroepithelial tumor

Pathology

  • WHO grade II
    • Higher grade atypical, anaplastic variant is rare
  • Round cells with stippled nuclei are characteristic microscopic features
  • Electron microscopy: synaptophysin-positive

Clinical Features

  • Affects all ages (commonly 3rd decade of life)
  • No gender predilection
  • Commonly presents with signs/symptoms of increased intracranial pressure secondary to obstructive hydrocephalus
    • Headache, nausea, vomiting, and altered mental status
  • Treatment: surgical resection; chemoradiation or radiosurgery for unresectable tumors
  • Prognosis: recurrence uncommon following total resection; 5-year survival 90%

Imaging Features

  • General
    • Supratentorial, intraventricular mass with cystic or bubbly appearance
    • Often located with lateral ventral frontal horn or body near the foramen of Monro
      • Attachment to the septum pellucidum or lateral ventricular wall
      • ± 3rd ventricular extension
    • Frequently shows calcification
  • CT

    • Heterogeneous density intraventricular mass due to solid and cystic components
    • ± Calcification
    • Heterogeneous enhancement on contrast-enhanced CT
  • MRI

    • T1WI: heterogeneous but predominantly isointense
    • T2WI: usually hyperintense with cystic or bubbly appearance; ± flow voids
    • FLAIR: heterogeneously hyperintense
    • T2*/GRE/SWI: black signal blooming in foci of calcification
    • T1WI+C: heterogeneous enhancement
    • MRS: increased Cho, decreased NAA; glycine peak at 3.55

Imaging Recommendations

  • MRI with contrast including coronal T2WI to better detect tumor attachment site

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Rachel Seltman, MD; Jacob A. Eitel, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.05

References

Donoho D, et al. Imaging of central neurocytomas. Neurosurg Clin N Am. 2015;26:11-19.

Kocaoglu M, et al. Central neurocytoma: proton MR spectroscopy and diffusion weighted MR imaging findings. Magn Reson Imaging. 2009;27:434-440.

Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.

Patel DM, et al. Update on the diagnosis, pathogenesis, and treatment strategies for central neurocytoma. J Clin Neurosci. 2013;20:1193-1199.

Schmidt MH, et al. Central neurocytoma: a review. J Neurooncol. 2004;66:377-384.

Tlili-Graiess K, et al. Diffusion weighted MR imaging and proton MR spectroscopy findings of central neurocytoma with pathological correlation. J Neuroradiol. 2014;41:243-250.

Yeh IB, et al. Central neurocytoma: typical magnetic resonance spectroscopy findings and atypical ventricular dissemination. Magn Reson Imaging. 2008;26:59-64.

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