CNS & pituitary tumors

Gliomas, glioneuronal tumors, and neuronal tumors

Neuronal and mixed neuronal-glial tumors

Central neurocytoma



Last author update: 2 June 2023
Last staff update: 11 October 2023

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PubMed Search: Central neurocytoma

Daniel D. Child, M.D., Ph.D.
Rebecca Yoda, M.D.
Page views in 2023: 11,360
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Cite this page: Child DD, Yoda R. Central neurocytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorcentralneurocytoma.html. Accessed April 14th, 2024.
Definition / general
  • Rare, well differentiated, intraventricular neoplasm with neuroepithelial differentiation, typically arising near the foramen of Monro
Essential features
Terminology
  • Central neurocytoma
ICD coding
  • ICD-O:
    • 9506/0 - central neurocytoma benign
    • 9506/1 - central neurocytoma
  • ICD-10:
    • D43.0 - neoplasm of uncertain behavior of brain, supratentorial
    • D43.1 - neoplasm of uncertain behavior of brain, infratentorial
    • D43.2 - neoplasm of uncertain behavior of brain, unspecified
    • D43.9 - neoplasm of uncertain behavior of central nervous system, unspecified
  • ICD-11:
    • 2A00.3 - central neurocytoma of brain
    • XH0C11 - central neurocytoma
Epidemiology
Sites
Pathophysiology
Etiology
  • Unknown at this time
Diagrams / tables

Images hosted on other servers:

Coronal section diagram

Clinical features
Diagnosis
  • Based primarily on histologic and immunophenotypic features (Brain Pathol 1993;3:297)
  • Requires intraventricular localization, oligodendroglioma-like cytology and synaptophysin expression
  • Correlation with radiologic studies (e.g., MRI, CT) is essential; supportive findings include
    • Intraventricular localization (required)
    • Enhancing, multicystic mass
    • MRI: T1 isointense, T2 heterogeneous, FLAIR hyperintense
    • CT: may show calcifications
  • Methylation profiling may aid diagnosis in unresolved cases (J Neurooncol 2022;159:725)
Radiology description
  • Noncontrast computed tomography (J Clin Neurosci 2013;20:679, Neurosurg Clin N Am 2015;26:11)
    • Highly variable appearance, often with mixed solid and cystic components that appear isodense and hypodense, respectively, to surrounding brain parenchyma
    • Calcifications may be seen, typically partial or punctate
    • Evidence of hydrocephalus or hemorrhage may be visible
  • Magnetic resonance imaging (J Clin Neurosci 2012;19:681, J Clin Neurosci 2013;20:679, Neurosurg Clin N Am 2015;26:11)
    • Classically an intraventricular mass with a multicystic, soap bubble appearance characterized by T1 and T2 isointense solid components and T2 hyperintense, fluid filled cysts
    • Peripheral cyst walls may form spicules and cause undulation of the adjacent lateral ventricle wall (scalloping)
    • Calcifications and flow voids may be seen on T1 sequences
    • Heterogenous contrast enhancement
    • No surrounding peritumoral edema on T2 / FLAIR
  • Proton magnetic resonance spectroscopy (Eur Radiol 2009;19:2049, J Clin Neurosci 2012;19:681, Neurosurg Clin N Am 2015;26:11)
    • Characteristic glycine peak at 3.55 ppm
    • Prominent choline peak
    • Inverted alanine peak
Radiology images

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Intraventricular mass on MRI

Calcifications on CT

T1+C MRI

Prognostic factors
Case reports
Treatment
Clinical images

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Endoscopic view of intraventricular tumor

Gross description
Gross images

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Intraventricular mass

Frozen section description
  • Sheets of isomorphous, round cells in a fibrillary background
  • Minimal pleomorphism
  • Necrosis or mitotic figures are typically absent
  • Reference: Acta Cytol 2004;48:194
Intraoperative frozen / smear cytology images

Contributed by Rebecca Yoda, M.D.

Cells with monotonous round nuclei

Intraoperative squash preparation

Microscopic (histologic) description
  • Neuroepithelial neoplasm composed of uniform, small - medium cells growing in sheets with indistinct cytoplasm (Brain Pathol 1993;3:297)
    • Nuclei are round with regular contours, finely stippled (salt and pepper) chromatin and micronucleoli
    • Perinuclear clearing may be prominent (similar to oligodendrogliomas)
    • Arborizing capillaries
    • Large hyalinized blood vessels
    • Other morphologic features may include
      • Honeycomb-like architecture
      • Patches of fibrillar, neuropil-like matrix mimicking pineocytomatous rosettes
      • Perivascular pseudorosettes
      • Homer-Wright rosettes
      • Ganglioid cells
    • Calcification is usually distributed throughout the tumor and may be prominent
  • Lipomatous differentiation occurs rarely (World Neurosurg 2018;120:214)
  • Hemorrhage is sometimes present; hemosiderin laden macrophages may be seen (Neurosurg Rev 2001;24:48)
  • Designated as atypical central neurocytoma when anaplastic features are seen (J Neurosurg 1992;76:32, Brain Pathol 1993;3:297)
    • Brisk mitotic activity
    • Microvascular proliferation
    • Necrosis
Microscopic (histologic) images

Contributed by Rebecca Yoda, M.D.

Circumscribed border

Fibrillary matrix

Perinuclear clearing

Perivascular pseudorosette

Neurocytic rosettes


Linear arrangement

Calcifications

Branching capillaries

Hyalinized vessels

Lipomatous change


Necrosis

Mitotic activity

Synaptophysin IHC

NeuN IHC

GFAP IHC



Contributed by Nazar M. T. Jawhar, M.D. (Case #119)

Circumscribed border

Perivascular pseudorosettes

Perinuclear clearing

Rosettes and perivascular pseudorosettes


Calcification

Linear cellular arrangement

Uniform, round cells

Virtual slides

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Left lateral ventricle tumor

Cytology description
  • Cerebral spinal fluid cytology may be positive in the presence of disseminated tumor (J Neurosurg 1992;76:32)
    • Crowded, cellular spheres composed of uniform small - medium cells
    • Scant, cyanophilic cytoplasm on Papanicolau stain
    • Neurocytic rosettes may be seen
  • Squash preparations / direct smears (Acta Cytol 2004;48:194, Acta Cytol 2010;54:209)
    • Monotonous, round cells with ill defined cytoplasm and without aggregation or clustering
    • Nuclei tend to have finely granular chromatin and micronucleoli
    • Hemosiderin laden macrophages or reactive astrocytes may be present
Electron microscopy description
Electron microscopy images

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TEM showing neuronal features

Ultrastructure recapitulating neuropil and synapses

Molecular / cytogenetics description
Molecular / cytogenetics images

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DNA methylation based classification

Sample pathology report
  • Brain, intraventricular mass, resection:
    • Central neurocytoma, CNS WHO grade 2
Differential diagnosis
Board review style question #1

A 29 year old woman with an intraventricular brain mass is diagnosed with a central neurocytoma with histologic findings shown above. Which of the following immunohistochemical stains is most likely to be diffusely positive in this neoplasm?

  1. Chromogranin
  2. GFAP
  3. Olig2
  4. Synaptophysin
Board review style answer #1
D. Synaptophysin. Synaptophysin expression is the most reliable diagnostic marker for central neurocytoma, which generally shows low to absent immunoreactivity for chromogranin, GFAP and Olig2.

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Reference: Central neurocytoma
Board review style question #2
Which of the following histological features is necessary for the designation of atypical central neurocytoma?

  1. Hypercellularity
  2. Lipomatous differentiation
  3. Macronucleoli
  4. Microvascular proliferation
Board review style answer #2
D. Microvascular proliferation: Brisk mitotic activity, necrosis and microvascular proliferation are the 3 histological features necessary for the classification of an atypical central neurocytoma. Answers A and C are incorrect because hypercellularity and macronucleoli are 2 of several criteria used for the diagnosis of an atypical meningioma but are not used to evaluate for atypical central neurocytoma. Answer B is incorrect because lipomatous differentiation may be seen in rare cases of central neurocytoma but does not confer an atypical designation.

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Reference: Central neurocytoma
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