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| | Tx = | | | Tx = |
| }} | | }} |
| :'''Not''' to be confused with ''[[ganglioneuroma]]''.
| | 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. |
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| ==Imaging== | | ==Imaging== |
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| *Strong CM enhancement. | | *Strong CM enhancement. |
| *May contain cysts. | | *May contain cysts. |
| *Associated with midline structures. | | *Associated with temporal lobe. |
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| ==Gross== | | ==Gross== |
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| ==Microscopic== | | ==Microscopic== |
| Features:<ref name=Ref_PSNP82-4>{{Ref PSNP|82-4}}</ref>
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| *Classically biphasic (though either may be absent):
| | ====Microscopic==== |
| *#Fibrillar. | | Features: |
| *#Microcystic/loose. | | *Dysplastic neurons. |
| *Hair-like fibres ~ 1 micrometer; ''pilo-'' = hair.<ref>URL: [http://dictionary.reference.com/browse/pilo- http://dictionary.reference.com/browse/pilo-]. Accessed on: 24 November 2010.</ref> | | **Out of regular architecture / abnormal location. |
| **Best seen on smear or with GFAP [[IHC]]. | | **Cytomegaly |
| *Rosenthal fibres - '''key feature'''. | | **Clustering |
| **May be rare. Not pathognomonic (see below). | | **Binucleated (very occassionally). |
| | *Atypical glia. |
| *Eosinophilic granular bodies. | | *Eosinophilic granular bodies. |
| *Low cellularity - when compared to medulloblastoma and ependymoma. | | *Calcification. |
| | *Prominent capillary network. |
| | *Lymphocytic cuffing. |
| | *May contain some reticulin. |
| | *Glial component may resemble: |
| | **Fibrillary astrocytoma. |
| | **Oligodendroglioma. |
| | **Pilocytic astrocytoma. |
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| Notes:
| | Anaplastic ganglioglioma: |
| *+/-Microvascular proliferation. | | *Brisk mitotic activity |
| *+/-Focal necrosis.
| | *Necrosis |
| **Necrosis with pseudopalisading more likely glioblastoma.
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| *+/-Mitoses - not significant in the context of the Dx.
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| DDx (of Rosenthal fibers):<ref>Munoz D. 9 Mar 2009.</ref>
| | ====IHC==== |
| *Chronic reactive gliosis. | | *Neurons: |
| *Subependymoma. | | **[[MAP2]] +ve |
| *Pilocytic astrocytoma. | | **Synaptophysin +ve |
| *Ganglioglioma. | | ** Neurofilament +ve |
| | *Glia: |
| | **CD34+/-ve |
| | *BRAF V600E +ve (approx. 25%, mainly ganglion cells). |
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| DDx of pilocystic astrocytoma (brief):
| | ====Molecular==== |
| *Piloid gliosis (esp. in sellar lesions). | | *BRAF V600E-mutated(approx. 25%). |
| *[[Oligodendroglioma]]. | | *IDH1/2 wt. |
| *[[Glioblastoma]] (uncommon - but important). | | *No 1p/19q codeletion. |
| *Tanycytic [[Ependymoma]] | | *Usu. Chr. 7 gain. |
| *Pilocytic tumor components may be found in [[Ganglioglioma]], [[DNET]], [[RGNT]] | | *CDKN2A deletions in anaplastic ganglioglioma. |
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| ===Images=== | | ===Images=== |
| ====Smears====
| | |
| <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> |
| ====Sections====
| |
| <gallery>
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| Image:Rosenthal_HE_40x.jpg | Rosenthal fibres. (WC)
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| Image:Pilocytic astrocytoma cell pleomorphism.jpg | Occasional pleomorphism. (WC)
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| Image:Pilocytic astrocytoma endothelial proliferations.jpg | Microvascular proliferation. (WC)
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| </gallery>
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| www:
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| *[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q19-Ans.htm Rosenthal fibre (ouhsc.edu)].
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| *[http://path.upmc.edu/cases/case162.html Pilocytic astrocytoma (upmc.edu)].
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| *[http://path.upmc.edu/cases/case90.html Pilocytic astrocytoma - another case (upmc.edu)].
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| *[http://path.upmc.edu/cases/case195/images/figure3b.jpg Pilocytic astrocytoma - pennies on a plate (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case195.html http://path.upmc.edu/cases/case195.html]. Accessed on: 8 January 2012.</ref>
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| *[http://path.upmc.edu/cases/case397.html Pilocytic astrocytoma (upmc.edu)].
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| ==Stains== | | ==Prognosis== |
| *PAS-D: eosinophilic granular bodies +ve. | | *Good (10-year OS: 90%), but epilepsy may continue. |
| | *Primary treatment: surgery. |
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| ==IHC== | | ====DDx:==== |
| Features:<ref name=Ref_PSNP84>{{Ref PSNP|84}}</ref>
| | *[[DNT]]. |
| *GFAP +ve (fibres).
| | *[[Oligodendroglioma]]. |
| *CD68: may have a significant macrophage component.
| | *Trapped cortical neurons in diffuse astrocytoma. |
| *KI-67: may be "high" (~20% ???).
| | *Papillary glioneuronal tumor. |
| *Olig 2: Usually strongly present.<ref name=pmid21193945>{{Cite journal | last1 = Otero | first1 = JJ. | last2 = Rowitch | first2 = D. | last3 = Vandenberg | first3 = S. | title = OLIG2 is differentially expressed in pediatric astrocytic and in ependymal neoplasms. | journal = J Neurooncol | volume = 104 | issue = 2 | pages = 423-38 | month = Sep | year = 2011 | doi = 10.1007/s11060-010-0509-x | PMID = 21193945 }}</ref>
| | *Dysembryoplastic neuroepithelial tumor. |
| *[[IDH1]] (R132H) -ve. | |
| *[[H3F3A]] (K27M) -ve. | |
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| ==Molecular==
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| * Almost all alteration associated with the MAPK pathway.<ref>{{Cite journal | last1 = Collins | first1 = VP. | last2 = Jones | first2 = DT. | last3 = Giannini | first3 = C. | title = Pilocytic astrocytoma: pathology, molecular mechanisms and markers. | journal = Acta Neuropathol | volume = 129 | issue = 6 | pages = 775-88 | month = Jun | year = 2015 | doi = 10.1007/s00401-015-1410-7 | PMID = 25792358 }}</ref>
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| * KIAA1549-BRAF fusion transcripts most common in sporadic PA (up to 2/3 of all cases).
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| **DDx: Fusion reported in rare Diffuse Leptomeingeal Glioneuronal Tumors and Oligodendroglioma.
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| * Rarely BRAF, KRAS or FGFR1 mutations, NTRK2, SRGAP3-RAF1 or FAM131B-BRAF fusions.<ref>{{Cite journal | last1 = Jones | first1 = DT. | last2 = Hutter | first2 = B. | last3 = Jäger | first3 = N. | last4 = Korshunov | first4 = A. | last5 = Kool | first5 = M. | last6 = Warnatz | first6 = HJ. | last7 = Zichner | first7 = T. | last8 = Lambert | first8 = SR. | last9 = Ryzhova | first9 = M. | title = Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. | journal = Nat Genet | volume = 45 | issue = 8 | pages = 927-32 | month = Aug | year = 2013 | doi = 10.1038/ng.2682 | PMID = 23817572 }}</ref><ref>{{Cite journal | last1 = Cin | first1 = H. | last2 = Meyer | first2 = C. | last3 = Herr | first3 = R. | last4 = Janzarik | first4 = WG. | last5 = Lambert | first5 = S. | last6 = Jones | first6 = DT. | last7 = Jacob | first7 = K. | last8 = Benner | first8 = A. | last9 = Witt | first9 = H. | title = Oncogenic FAM131B-BRAF fusion resulting from 7q34 deletion comprises an alternative mechanism of MAPK pathway activation in pilocytic astrocytoma. | journal = Acta Neuropathol | volume = 121 | issue = 6 | pages = 763-74 | month = Jun | year = 2011 | doi = 10.1007/s00401-011-0817-z | PMID = 21424530 }}</ref>
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| *Up to 15% of all [[NF1]] patients develop a PA ("optic glioma" as predilection).<ref>{{Cite journal | last1 = Friedrich | first1 = RE. | last2 = Nuding | first2 = MA. | title = Optic Pathway Glioma and Cerebral Focal Abnormal Signal Intensity in Patients with Neurofibromatosis Type 1: Characteristics, Treatment Choices and Follow-up in 134 Affected Individuals and a Brief Review of the Literature. | journal = Anticancer Res | volume = 36 | issue = 8 | pages = 4095-121 | month = Aug | year = 2016 | doi = | PMID = 27466519 }}</ref>
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| *Rare reports of PA in Noonan-Syndrome (PTPN11 mutation).<ref>{{Cite journal | last1 = Jones | first1 = DT. | last2 = Hutter | first2 = B. | last3 = Jäger | first3 = N. | last4 = Korshunov | first4 = A. | last5 = Kool | first5 = M. | last6 = Warnatz | first6 = HJ. | last7 = Zichner | first7 = T. | last8 = Lambert | first8 = SR. | last9 = Ryzhova | first9 = M. | title = Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. | journal = Nat Genet | volume = 45 | issue = 8 | pages = 927-32 | month = Aug | year = 2013 | doi = 10.1038/ng.2682 | PMID = 23817572 }}</ref> | |
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| ==Prognosis==
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| *Excellent (10-year OS: 90%) | |
| *In thalamic/chiasmatic region not so good (incomplete resection, often [[Pilomyxoid astrocytoma]]).
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| *Primary treatment: surgery. Incomplete resection: RT has to be considered. | |
| **Chx is given in rare cases that are still progredient<ref>{{Cite journal | last1 = Metts | first1 = RD. | last2 = Bartynski | first2 = W. | last3 = Welsh | first3 = CT. | last4 = Kinsman | first4 = S. | last5 = Bredlau | first5 = AL. | title = Bevacizumab Therapy for Pilomyxoid Astrocytoma. | journal = J Pediatr Hematol Oncol | volume = | issue = | pages = | month = Mar | year = 2017 | doi = 10.1097/MPH.0000000000000824 | PMID = 28338567 }}</ref>
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| ==See also== | | ==See also== |