CNS tumor
Neuronal and mixed neuronal-glial tumors
Gangliocytoma & ganglioglioma
Author: Eman Abdelzaher, M.D., Ph.D.
Topic Completed: 1 July 2013
Minor changes: 28 September 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Ganglion cell [title] tumors
Table of Contents
Definition / general | Epidemiology | Sites | Clinical features | Grading | Radiology description | Radiology images | Case reports | Gross description | Gross images | Microscopic (histologic) description | Cytology images | Positive stains | Electron microscopy description | Differential diagnosis | Additional referencesCite this page: Abdelzaher E. Gangliocytoma & ganglioglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorganglioncelltumor.html. Accessed September 30th, 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
Images hosted on other servers:
Gangliocytoma:
Ganglioglioma:
Anaplastic ganglioglioma:
Case reports
Gangliocytoma:
Ganglioglioma:
Anaplastic ganglioglioma:
- 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
Microscopic (histologic) description
General:
Gangliocytoma:
Ganglioglioma:
Anaplastic ganglioglioma:
- 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
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
