Difference between revisions of "Ganglioglioma"
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*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%) | *Favourable prognosis (survival rates up to 97%) | ||
** | **Anaplastic ganglioglioma have a recurrence risk of 69%<ref>{{Cite journal | last1 = Terrier | first1 = LM. | last2 = Bauchet | first2 = L. | last3 = Rigau | first3 = V. | last4 = Amelot | first4 = A. | last5 = Zouaoui | first5 = S. | last6 = Filipiak | first6 = I. | last7 = Caille | first7 = A. | last8 = Almairac | first8 = F. | last9 = Aubriot-Lorton | first9 = MH. | title = Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database. | journal = Neuro Oncol | volume = 19 | issue = 5 | pages = 678-688 | month = 05 | year = 2017 | doi = 10.1093/neuonc/now186 | PMID = 28453747 }}</ref> | ||
==Imaging== | ==Imaging== | ||
Revision as of 10:26, 14 September 2017
| Ganglioglioma | |
|---|---|
| Diagnosis in short | |
 | |
| LM DDx | piloid gliosis, pilocytic astrocytoma, DNT |
| Stains | PAS-D +ve (eosinophilic granular bodies) |
| IHC | GFAP +ve, Synapto +ve |
| Gross | usually temporal +/-cystic |
| Site | brain - usu. supratentorial |
|
| |
| Syndromes | associated with epilepsy |
|
| |
| Prevalence | rare - esp. in children |
| Prognosis | good (WHO Grade I) |
Ganglioglioma is a epilepsy-associated glioneuronal tumour with benign course. Not to be confused with ganglioneuroma.
General
- Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1).
- Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3)
- Rare (approx. 0.5% of all CNS tumors).
- Usu. temporal lobe.
- Predominantly children (mean age: 9 years).
- Recognized as a cause of epilepsy.[1]
- Favourable prognosis (survival rates up to 97%)
- Anaplastic ganglioglioma have a recurrence risk of 69%[2]
Imaging
- Well-defined, T2-hyperintense.
- Strong CM enhancement.
- May contain cysts.
- Associated with temporal lobe.
Gross
- Circumscribed lesion.
- Usu. contrast enhancing.
- Solid, but intracortical cysts may be present.
- Little mass effect.
Microscopic
Microscopic
Features:
- Dysplastic neurons.
- Out of regular architecture / abnormal location.
- Cytomegaly
- Clustering
- Binucleated (very occassionally).
- Atypical glia (ie neoplastic).
- Eosinophilic granular bodies (more common than rosenthal fibers).
- Dystrophic 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
- Chromogranin +ve
- Glia:
- CD34+/-ve
- BRAF V600E +ve (approx. 25%, mainly ganglion cells).
- MAP2: usu. absent.
- MIB-1 (low, but resembles proliferative tumor component).
Molecular
- BRAF V600E-mutated(approx. 25%).[3]
- BRAF V600E antibody stains especially neuronal cells.[4]
- IDH1/2 wt.
- No 1p/19q codeletion.
- Usu. Chr. 7 gain.
- CDKN2A deletions in anaplastic ganglioglioma.
Images
Lymphocytic cuffing in ganglioglioma (WC/jensflorian)
Calcification in ganglioglioma (WC/jensflorian)
CD34 immunostain in ganglioglioma (WC/jensflorian)
Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian)
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
References
- ↑ Im, SH.; Chung, CK.; Cho, BK.; Lee, SK. (Mar 2002). "Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome.". J Neurooncol 57 (1): 59-66. PMID 12125968.
- ↑ Terrier, LM.; Bauchet, L.; Rigau, V.; Amelot, A.; Zouaoui, S.; Filipiak, I.; Caille, A.; Almairac, F. et al. (05 2017). "Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database.". Neuro Oncol 19 (5): 678-688. doi:10.1093/neuonc/now186. PMID 28453747.
- ↑ Schindler, G.; Capper, D.; Meyer, J.; Janzarik, W.; Omran, H.; Herold-Mende, C.; Schmieder, K.; Wesseling, P. et al. (Mar 2011). "Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma.". Acta Neuropathol 121 (3): 397-405. doi:10.1007/s00401-011-0802-6. PMID 21274720.
- ↑ Koelsche, C.; Wöhrer, A.; Jeibmann, A.; Schittenhelm, J.; Schindler, G.; Preusser, M.; Lasitschka, F.; von Deimling, A. et al. (Jun 2013). "Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells.". Acta Neuropathol 125 (6): 891-900. doi:10.1007/s00401-013-1100-2. PMID 23435618.