Difference between revisions of "Ganglioglioma"
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Ganglioglioma is a epilepsy-associated glioneuronal tumour with benign course. '''Not''' to be confused with ''[[ganglioneuroma]]''. | |||
====General==== | ====General==== | ||
*Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1). | *Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1). | ||
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*Favourable prognosis (survival rates up to 97%) | *Favourable prognosis (survival rates up to 97%) | ||
**Insufficient data für anaplastic ganglioglioma. | **Insufficient data für anaplastic ganglioglioma. | ||
==Imaging== | ==Imaging== | ||
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*Strong CM enhancement. | *Strong CM enhancement. | ||
*May contain cysts. | *May contain cysts. | ||
*Associated with | *Associated with temporal lobe. | ||
==Gross== | ==Gross== | ||
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==Microscopic== | ==Microscopic== | ||
====Microscopic==== | |||
* | Features: | ||
* | *Dysplastic neurons. | ||
* | **Out of regular architecture / abnormal location. | ||
** | **Cytomegaly | ||
* | **Clustering | ||
** | **Binucleated (very occassionally). | ||
*Atypical glia. | |||
*Eosinophilic granular bodies. | *Eosinophilic granular bodies. | ||
* | *Calcification. | ||
*Prominent capillary network. | |||
*Lymphocytic cuffing. | |||
*May contain some reticulin. | |||
*Glial component may resemble: | |||
**Fibrillary astrocytoma. | |||
**Oligodendroglioma. | |||
**Pilocytic astrocytoma. | |||
Anaplastic ganglioglioma: | |||
* | *Brisk mitotic activity | ||
*Necrosis | |||
====IHC==== | |||
* | *Neurons: | ||
* | **[[MAP2]] +ve | ||
* | **Synaptophysin +ve | ||
* | ** Neurofilament +ve | ||
*Glia: | |||
**CD34+/-ve | |||
*BRAF V600E +ve (approx. 25%, mainly ganglion cells). | |||
====Molecular==== | |||
* | *BRAF V600E-mutated(approx. 25%). | ||
* | *IDH1/2 wt. | ||
* | *No 1p/19q codeletion. | ||
* | *Usu. Chr. 7 gain. | ||
* | *CDKN2A deletions in anaplastic ganglioglioma. | ||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian) | File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian) | ||
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File:Anaplastic ganglioglioma HE.jpg | Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian) | File:Anaplastic ganglioglioma HE.jpg | Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian) | ||
</gallery> | </gallery> | ||
== | ==Prognosis== | ||
* | *Good (10-year OS: 90%), but epilepsy may continue. | ||
*Primary treatment: surgery. | |||
== | ====DDx:==== | ||
*[[DNT]]. | |||
*[[Oligodendroglioma]]. | |||
*Trapped cortical neurons in diffuse astrocytoma. | |||
*Papillary glioneuronal tumor. | |||
*Dysembryoplastic neuroepithelial tumor. | |||
*[[ | |||
*[[ | |||
* | |||
* | |||
* | |||
==See also== | ==See also== |