CNS tumor
Neuronal and mixed neuronal-glial tumors
Central neurocytoma


Topic Completed: 1 January 2006

Minor changes: 28 September 2020

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PubMed Search: "Central neurocytoma" CNS

Nat Pernick, M.D.
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Cite this page: Pernick N. Central neurocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorcentralneurocytoma.html. Accessed October 20th, 2020.
Definition / general
  • First described in 1982 (Acta Neuropathol 1982;56:151)
  • Grows from foramen of Monro or septum pellucidum into lateral or third ventricle; often calcified
  • Most common neoplasm of septum pellucidum in young adults but still < 1% of all CNS tumors
  • Often presents with increased intracranial pressure due to obstructive hydrocephalus
  • Similar tumors within CNS parenchyma are called extraventricular neurocytoma
  • Rare before age 10 years
Case reports
Treatment
Gross description
  • Well demarcated from surrounding tissue
  • Gray, friable, may be gritty due to microcalcifications
Gross images

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Ventricular mass

Microscopic (histologic) description
  • Well developed neuronal features - monotonous bland cells with modest cytoplasm, often empty appearing "halo" resembling oligodendroglioma, salt and pepper chromatin, embedded in eosinophilic fibrillar matrix with rare Homer Wright rosettes and ganglion cells
  • Usually no infiltrating margin, no / rare necrosis, no endothelial proliferation and no / rare mitotic figures
Microscopic (histologic) images

Case #119


Various images

Cytology description
  • Cellular; monotonous round cells with ill defined cytoplasm, oval nuclei, finely granular chromatin and micronucleoli
  • Background fibrillar matrix; variable histiocytic giant cells with hemosiderin (Acta Cytol 2004;48:194)
Negative stains
  • GFAP (reactive astrocytes are GFAP+), Ki67 ( < 2%)
Electron microscopy description
  • Microtubules, 100 - 200 nm dense core vesicles, clear vesicles and neuritic type processes
  • Usually no intermediate filaments
Differential diagnosis
  • Clear cell ependymoma: negative for neuronal markers, strongly positive for GFAP, vimentin and EMA
  • Glioma: no / few halo cells, no / focal neuronal features and strongly GFAP+
  • Oligodendroglioma: no prominent neuronal features, negative or focal for neuronal markers
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