Ganglion cell tumors

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

Author: Eman Abdelzaher, M.D., Ph.D.

Topic Completed: 1 July 2013

Revised: 2 January 2019, last major update July 2013

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Ganglion cell [title] tumors

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Table of Contents

Cite this page: Abdelzaher E. Ganglion cell tumors. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorganglioncelltumor.html. Accessed August 22nd, 2019.

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

Images hosted on other servers:

Gangliocytoma:

Ganglioglioma:

Temporal lobe tumor

Various images

Anaplastic ganglioglioma:

Case reports

Gangliocytoma:

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

Ganglioglioma:

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: Various Sensory Abnormalities)
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: Brain Pathology Case of the Month)
Various cases with combined pleomorphic xanthoastrocytoma (Arch Pathol Lab Med 2000;124:1707, Am J Surg Pathol 1997;21:763)

Anaplastic ganglioglioma:

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

Solid or cystic
No hemorrhage or necrosis

Gross images

Images hosted on other servers:

Gangliocytoma:

Hypothalamo - infundibular region

Ganglioglioma:

Various images

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

Images hosted on other servers:

Gangliocytoma:

Nerve cells of variable size and shape

Tumor attached to dura (fig 1e)

Intrasellar tumor

Synaptophysin+ (fig 1f)

Ganglioglioma:

Large neurons

Reticulin stain

Variable sized astrocytes, nerve cells and calcification

Nesting pattern with delicate vasculature

Various images

Tumor with neurofibrillary tangles

Large, mature neurons + / - glial component

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

Anaplastic ganglioglioma:

Various images

Pleomorphic ganglion cells

Cytology images

Images hosted on other servers:

Ganglioglioma:

Ganglion cell with prominent nucleoli and neoplastic astrocytes

Positive stains

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

Electron microscopy description

Neurons with dense core granules are characteristic and diagnostically useful

Differential diagnosis

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, nonenhancing
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

Al Barbarawi: Neuronal and Mixed Neuronal - Glial Tumors of the Central Nervous System, 2012, Radiographics 2001;21:1533, Radiopaedia: Ganglioglioma