Definition
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• Well differentiated neuroepithelial tumors composed of neoplastic ganglion cells (gangliocytoma) or combination of neoplastic ganglion cells and neoplastic glial component (ganglioglioma)

Epidemiology
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• <2% of all brain tumors
• Ages 2 months to 70 years, with predilection for children and young adults

Sites
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• Throughout CNS, but >70% involve the temporal lobe

Grading
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• Gangliocytoma, WHO grade I
• Ganglioglioma, WHO grade I
• Anaplastic ganglioglioma, WHO grade III
• Criteria for grade II are not yet established

Clinical features
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• Associated with long term epilepsy

Radiology
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• Circumscribed solid mass or cyst with a mural nodule
• Variable enhancement, variable calcification

Radiology images
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Temporal lobe tumor

Case reports
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• 48 year old woman with acromegaly and intrasellar tumor (Arch Pathol Lab Med 2005;129:415)

• Infant with brainstem ganglioglioma (J Pediatr Neurosci 2013;8:41)
• 4 year old boy with seizures (Arch Pathol Lab Med 2003;127:e387)
• 30 year old man with spinal tumor (University of Pittsburgh Case 142)
• 36 year old woman with coexisting dysembryoplastic neuroepithelial tumor (Arch Pathol Lab Med 1999;123:247)
• 51 year old man with ganglioglioma containing neurofibrillary tangles (University of Pittsburgh Case 66)
• Various cases with combined pleomorphic xanthoastrocytoma (Arch Pathol Lab Med 2000;124:1707, Am J Surg Pathol 1997;21:763)

• 6 year old girl with composite grade III/IV ganglioglioma and giant cell glioblastoma (Arch Pathol Lab Med 1999;123:342)
• 7 year old girl with dissemination to spinal cord (Surg Neurol 1998;49:445)
• 45 year old woman with 5 month history of headache and dizziness (J Korean Med Sci 2007;22:S139)
• 53 year old woman with metastasis through a ventriculoperitoneal shunt (Pathol Int 1999;49:258)
• 59 year old man with sarcomatous component (Neuropathology 2002;22:40)

Gross description
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• Solid or cystic
• No hemorrhage or necrosis

Gross images
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Hypothalamo-infundibular region

Various images

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

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

• Neoplastic ganglion cells
• Neoplastic glial component:
  • Constitutes the proliferative component of the tumor (Ki-67 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 changes involving the glial component
  • Increased mitotic activity
  • Vascular proliferation
  • Necrosis
• Anaplastic changes in neuronal component are rare

Micro images
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Nerve cells of variable size and shape

Mature neurons with prominent nucleoli

Tumor attached to dura (arrow)

Intrasellar tumor from
above case history
composed of ganglion cells
and adenohypophyseal cells

Synaptophysin+ cytoplasm and cell processes

     

Mildly atypical glial cells and neurons

Variable sized astrocytes, nerve cells and calcification

Nesting pattern with delicate vasculature

Various images

Tumor with neurofibrillary tangles

Large, mature neurons +/- glial component

Various images

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+

 

Synaptophysin

 

GFAP+ astrocytes and their processes

Various images

Pleomorphic ganglion cells

Cytology images
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Ganglion cell with prominent nucleoli and neoplastic astrocytes

Positive stains
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• Ganglion cells: synaptophysin+, chromogranin+, NeuN+/-, neurofilament+, MAP2+, CD34+ (70-80%)
• Glial component: GFAP+

Electron microscopy description
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• Neurons with dense core granules are characteristic and diagnostically useful

Differential diagnosis
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• Cortical dysplasia and cortical tubers: no cyst or compact architecture
• Desmoplastic infantile ganglioglioma: markedly desmoplastic, smaller ganglion cells
• Dysplastic cerebellar gangliocytoma: site specific, associated with Cowden syndrome
• Hypothalamic hamartoma: classic location, non-enhancing
• Infiltrating glioma with entrapped neurons: no abnormal clustering or binucleation of the entrapped neurons
• Normal brain: organized, no dysplastic neurons
• Pilocytic astrocytoma: no neoplastic neurons
• Pleomorphic xanthoastrocytoma: pleomorphic astrocytes, +/- lipidization
• Subependymal giant cell astrocytoma: site specific, associated with tuberous sclerosis

Additional references
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• Neuronal and Mixed Neuronal-Glial Tumors of the Central Nervous System, Radiographics 2001;21:1533, Radiopaedia

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