Difference between revisions of "Ganglioglioma"

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:'''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).
Line 40: Line 41:
*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 midline structures.
*Associated with temporal lobe.


==Gross==
==Gross==
Line 56: Line 55:


==Microscopic==
==Microscopic==
Features:<ref name=Ref_PSNP82-4>{{Ref PSNP|82-4}}</ref>
 
*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.


Notes:  
Anaplastic ganglioglioma:
*+/-Microvascular proliferation.
*Brisk mitotic activity
*+/-Focal necrosis.
*Necrosis
**Necrosis with pseudopalisading more likely glioblastoma.
*+/-Mitoses - not significant in the context of the Dx.


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).


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.


===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)
Line 94: Line 102:
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>
Image:Rosenthal_HE_40x.jpg | Rosenthal fibres. (WC)
Image:Pilocytic astrocytoma cell pleomorphism.jpg | Occasional pleomorphism. (WC)
Image:Pilocytic astrocytoma endothelial proliferations.jpg | Microvascular proliferation. (WC)
</gallery>
www:
*[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q19-Ans.htm Rosenthal fibre (ouhsc.edu)].
*[http://path.upmc.edu/cases/case162.html Pilocytic astrocytoma (upmc.edu)].
*[http://path.upmc.edu/cases/case90.html Pilocytic astrocytoma - another case (upmc.edu)].
*[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>
*[http://path.upmc.edu/cases/case397.html Pilocytic astrocytoma (upmc.edu)].


==Stains==
==Prognosis==
*PAS-D: eosinophilic granular bodies +ve.
*Good (10-year OS: 90%), but epilepsy may continue.
*Primary treatment: surgery.  


==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.
 
==Molecular==
* 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>
* KIAA1549-BRAF fusion transcripts most common in sporadic PA (up to 2/3 of all cases).
**DDx: Fusion reported in rare Diffuse Leptomeingeal Glioneuronal Tumors and Oligodendroglioma.
* 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>
*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>
*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>
 
==Prognosis==
*Excellent (10-year OS: 90%)
*In thalamic/chiasmatic region not so good (incomplete resection, often [[Pilomyxoid astrocytoma]]).
*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>


==See also==
==See also==

Revision as of 10:10, 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%)
    • Insufficient data für anaplastic ganglioglioma.

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.
  • 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

  • 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

  1. ↑ 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.
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