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→Ganglioglioma: update (WHO 2016 compatible)
Jensflorian (talk | contribs) (→Table of common gliomas - grading: moved to glioma) |
Jensflorian (talk | contribs) (→Ganglioglioma: update (WHO 2016 compatible)) |
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:'''Not''' to be confused with ''[[ganglioneuroma]]''. | :'''Not''' to be confused with ''[[ganglioneuroma]]''. | ||
====General==== | ====General==== | ||
*Grade I WHO mixed neuronal-glial tumour | *Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1). | ||
*Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3) | |||
*Rare. | *Rare (approx. 0.5% of all CNS tumors). | ||
*Usu. temporal lobe. | *Usu. temporal lobe. | ||
*Predominantly children (mean age: 9 years). | |||
*Recognized as a cause of [[epilepsy]].<ref name=pmid12125968>{{Cite journal | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi = | PMID = 12125968 }}</ref> | *Recognized as a cause of [[epilepsy]].<ref name=pmid12125968>{{Cite journal | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi = | PMID = 12125968 }}</ref> | ||
*Favourable prognosis (survival rates up to 97%) | |||
**Insufficient data für anaplastic ganglioglioma. | |||
====Macroscopic==== | |||
*Circumscribed lesion. | |||
*Usu. contrast enhancing. | |||
*Solid, but intracortical cysts may be present. | |||
*Little mass effect. | |||
====Microscopic==== | ====Microscopic==== | ||
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**Binucleated (very occassionally). | **Binucleated (very occassionally). | ||
*Atypical glia. | *Atypical glia. | ||
*Eosinophilic granular bodies. | |||
*Calcification. | *Calcification. | ||
*Prominent capillary network. | |||
*Lymphocytic cuffing. | *Lymphocytic cuffing. | ||
*May contain some reticulin. | |||
*Glial component may resemble: | |||
**Fibrillary astrocytoma. | |||
**Oligodendroglioma. | |||
**Pilocytic astrocytoma. | |||
Anaplastic ganglioglioma: | Anaplastic ganglioglioma: | ||
*Brisk mitotic activity | *Brisk mitotic activity | ||
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*Glia: | *Glia: | ||
**CD34+/-ve | **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. | |||
====DDx:==== | ====DDx:==== | ||
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*[[Oligodendroglioma]]. | *[[Oligodendroglioma]]. | ||
*Trapped cortical neurons in diffuse astrocytoma. | *Trapped cortical neurons in diffuse astrocytoma. | ||
*Papillary glioneuronal tumor. | |||
*Dysembryoplastic neuroepithelial tumor. | |||
====Images==== | ====Images==== | ||
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File:Ganglioglioma calcification.jpg | Calcification in ganglioglioma (WC/jensflorian) | File:Ganglioglioma calcification.jpg | Calcification in ganglioglioma (WC/jensflorian) | ||
File:Ganglioglioma Cd34 x200.jpg | CD34 immunostain in ganglioglioma (WC/jensflorian) | File:Ganglioglioma Cd34 x200.jpg | CD34 immunostain in ganglioglioma (WC/jensflorian) | ||
File:Anaplastic ganglioglioma HE.jpg | Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian) | |||
</gallery> | </gallery> | ||
*[http://path.upmc.edu/cases/case142.html Ganglioglioma - case 1 (upmc.edu)]. | *[http://path.upmc.edu/cases/case142.html Ganglioglioma - case 1 (upmc.edu)]. | ||
*[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)]. | *[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)]. | ||
===Lhermitte-Duclos disease=== | ===Lhermitte-Duclos disease=== | ||