CNS tumor
Neuronal and mixed neuronal - glial tumors
Ganglion cell tumors


Topic Completed: 1 July 2013

Minor changes: 16 April 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Ganglion cell [title] tumors

Eman Abdelzaher, M.D., Ph.D.
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Cite this page: Abdelzaher E. Ganglion cell tumors. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorganglioncelltumor.html. Accessed May 29th, 2020.
Definition / general
  • Well differentiated neuroepithelial tumors composed of neoplastic ganglion cells (gangliocytoma) or combination of neoplastic ganglion cells and neoplastic glial component (ganglioglioma)
Epidemiology
  • < 2% of all brain tumors
  • Ages 2 months to 70 years, with predilection for children and young adults
Sites
  • Throughout CNS but > 70% involve the temporal lobe
Clinical features
  • Associated with long term epilepsy
Grading
  • Gangliocytoma, WHO grade I
  • Ganglioglioma, WHO grade I
  • Anaplastic ganglioglioma, WHO grade III
  • Criteria for grade II are not yet established
Radiology description
Neuroimaging:
  • Circumscribed solid mass or cyst with a mural nodule
  • Variable enhancement, variable calcification
Radiology images

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Gangliocytoma:


Ganglioglioma:


Anaplastic ganglioglioma:
Case reports
Gangliocytoma:

Ganglioglioma:

Anaplastic ganglioglioma:
Gross description
  • Solid or cystic
  • No hemorrhage or necrosis
Gross images

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Gangliocytoma:
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Hypothalamo - infundibular region

Microscopic (histologic) description
General:
  • Compact growth pattern
  • The hallmark of ganglion cell tumors is the presence of dysplastic / neoplastic ganglion cells that are identified by:
    • Loss of cytoarchitectural organization
    • Abnormal (subcortical) localization
    • Clustering
    • Large neurons (cytomegaly)
    • Coarse peripherally aggregated Nissl substance
    • Bi or multinucleated neurons with prominent nucleoli

Gangliocytoma:
  • Neoplastic ganglion cells
  • Stroma: finely fibrillar neuropil rich

Ganglioglioma:
  • Neoplastic ganglion cells
  • Neoplastic glial component:
    • Constitutes the proliferative component of the tumor (Ki67 is 1.1 to 2.7%)
    • Astrocytic: resemble fibrillary astrocytoma or pilocytic astrocytoma
    • Variable lobular architecture
    • Rare mitoses, no necrosis
  • Common features:
    • Perivascular lymphocytic cuffing
    • Rosenthal fibers and eosinophilic granular bodies
    • Microcysts
    • Rich reticulin network
    • Calcification
    • Glomeruloid vascular proliferation
    • Desmoplasia, especially with tumors involving the subarachnoid space

Anaplastic ganglioglioma:
  • Anaplastic changes involving the glial component
    • Increased mitotic activity
    • Vascular proliferation
    • Necrosis
  • Anaplastic changes in neuronal component are rare
Microscopic (histologic) images

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Gangliocytoma:
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Nerve cells of variable size and shape

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Tumor attached to dura (fig 1e)

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Intrasellar tumor

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Synaptophysin+ (fig 1f)



Ganglioglioma:
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Large neurons

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Reticulin stain

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Variable sized astrocytes, nerve cells and calcification

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Nesting pattern with delicate vasculature


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Various images

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Tumor with neurofibrillary tangles

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Large, mature neurons + / - glial component


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1: MRI shows temporal lobe mass with cystic component
2: Prominent granular bodies, Rosenthal fibers and microcalcifications
3: Like #2 plus large, binucleated ganglion cells and perivascular cuffing
4: NSE+


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Synaptophysin

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GFAP+ astrocytes and their processes



Anaplastic ganglioglioma:
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Various images

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Pleomorphic ganglion cells

Cytology images

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Ganglioglioma:
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Ganglion cell with prominent nucleoli and neoplastic astrocytes

Positive stains
Electron microscopy description
  • Neurons with dense core granules are characteristic and diagnostically useful
Differential diagnosis
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