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Neuropathology tumours (view source)

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#*[[Melanoma]].  
#*[[Melanoma]].  
#*[[Renal cell carcinoma]] (RCC).
#*[[Renal cell carcinoma]] (RCC).
# [[Glioblastoma]] (previously known as ''glioblastoma multiforme'').
# [[Glioblastoma]], IDH-wildtype.
# [[Anaplastic astrocytoma]].
# [[Astrocytoma, IDH-mutant]].
# [[Meningioma]].
# [[Meningioma]].


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# [[Medulloblastoma]].
# [[Medulloblastoma]].
# [[Ependymoma]].
# [[Ependymoma]].
# Pontine glioma, often [[Diffuse midline glioma, H3 K27-altered]].


===By location===
===By location===
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* [[Olfactory neuroblastoma]].
* [[Olfactory neuroblastoma]].
* [[Endolymphatic sac tumour]].
* [[Endolymphatic sac tumour]].
===Cystic tumours===
DDx:<ref>URL: [http://path.upmc.edu/cases/case320/dx.html http://path.upmc.edu/cases/case320/dx.html]. Accessed on: 14 January 2012.</ref>
*[[Pilocytic astrocytoma]].
*[[Pleomorphic xanthoastrocytoma]].
*[[Ganglioglioma]].
*[[Hemangioblastoma]].
*[[Craniopharyngioma]].<ref>URL: [http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral]. Accessed on: 14 January 2012.</ref>


===Primary versus secondary===
===Primary versus secondary===
*[[AKA]] (primary) brain tumour versus metastatic cancer.
*[[AKA]] (primary) brain tumour versus metastatic cancer.
====Primary====
====Primary====
Glial tumours:
[[Glioma|Glial tumours]]:
*Cytoplasmic processes - '''key feature'''.
*Cytoplasmic processes - '''key feature'''.
**Best seen at highest magnification - usu. ~1 micrometer.
**Best seen at highest magnification - usu. ~1 micrometer.
**Processes may branch.
**Processes may branch.
*Ill-defined border/blend with the surrounding brain.
*Ill-defined border/blend with the surrounding brain.
[[Meningioma]]:
*Lesion often dura-based.
*Mesenchymal tumor (often contains collagen).


[[Lymphoma]]:
[[Lymphoma]]:
*Primary CNS Lymphoma (PCNSL) is usu. a diffuse large B-cell lymphoma.
*Large (lymphoid) cells, ergo usu. not a difficult diagnosis.
*Large (lymphoid) cells, ergo usu. not a difficult diagnosis.
**~2x size of resting lymphocyte, nucleoli.
**~2x size of resting lymphocyte, nucleoli.
*Lesion predominantly perivascular.  
*Lesion predominantly perivascular.


====Secondary====
====Secondary====
Carcinomas:
*Carcinomas:
*Well-demarcated border between brain and lesion - '''key feature'''.
**Well-demarcated border between brain and lesion - '''key feature'''.
*No cytoplasmic processes.
**No cytoplasmic processes.
*Usu. have nuclear atypia of malignancy.
**Usu. have nuclear atypia of malignancy.
**Nuclei often ~3-4x the size of a [[RBC]].
**Nuclei often ~3-4x the size of a [[RBC]].
*+/-Glandular arrangement.
**+/-Glandular arrangement.
*+/-Nucleoli.
**+/-Nucleoli.
*Melanoma.
*Secondary Lymphoma.
*Sarcomas (rare).
 
===By growth pattern===
====Infiltrative astrocytomas====
*[[Astrocytoma, IDH-mutant]].
*[[Glioblastoma]], IDH-wildtype.
 
Notes:
**Glial: "blends into brain"/gradual transition to non-tumour brain.
 
====Non-infiltrative astrocytomas====
**[[Pilocytic astrocytoma]]
**[[Pleomorphic xanthoastrocytoma]]
**[[Subependymal giant cell astrocytoma]].
 
====Cystic tumours====
DDx:<ref>URL: [http://path.upmc.edu/cases/case320/dx.html http://path.upmc.edu/cases/case320/dx.html]. Accessed on: 14 January 2012.</ref>
*[[Pilocytic astrocytoma]].
*[[Pleomorphic xanthoastrocytoma]].
*[[Ganglioglioma]].
*[[Hemangioblastoma]].
*[[Craniopharyngioma]].<ref>URL: [http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral]. Accessed on: 14 January 2012.</ref>
 
 
Notes:
**Non-glial: no radiating glial processes.
*Rosenthal fibres within the tumour... often seen in [[pilocytic astrocytoma]].
**Rosenthal fibres may be seen around a (very) slow growing tumour and represent a reactive process.
*Inflammatory cells and macrophages should prompt consideration of an alternate diagnosis (e.g. [[cerebral infarct]], [[multiple sclerosis]]) - esp. if this is a primary lesion.<ref>URL: [http://path.upmc.edu/cases/case79/dx.html http://path.upmc.edu/cases/case79/dx.html]. Accessed on: 2 January 2012.</ref>
 
====Grading====
Nuclear pleomorphism present:
*At least grade II (diffuse astrocytoma).
 
Mitotic figures present:
*At least grade III (anaplastic astrocytoma).
 
Microvascular proliferation ''or'' necrosis with pseudopalisading tumour cells:
*Grade IV (glioblastoma [[AKA]] glioblastoma multiforme).
 
Notes:
*Pseudopalisading tumour cells = high tumour cell density adjacent to regions of necrosis; palisade = a fence of poles forming a defensive barrier or fortification.
*WHO Grading is currently based on expected biologiocal behaviour without treatment.
**Grading does not reflect molecular divergent groups within a tumor class  or response to therapy (Currently controversies in grading for IDH-mutant astrocytoma vs. IDH-wildtype astrocytoma).<ref>{{Cite journal  | last1 = Louis | first1 = DN. | last2 = von Deimling | first2 = A. | title = Grading of diffuse astrocytic gliomas: Broders, Kernohan, Zülch, the WHO… and Shakespeare. | journal = Acta Neuropathol | volume =  | issue =  | pages =  | month = Aug | year = 2017 | doi = 10.1007/s00401-017-1765-z | PMID = 28801693 }}</ref>
 
===By IHC===
*GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane) of most [[Astrocytoma]]s.
*[[IDH-1]](R132H) (isocitrate dehydrogenase 1) in [[Astrocytoma, IDH-mutant]].<ref name=pmid19228619>{{cite journal |author=Yan H, Parsons DW, Jin G, ''et al.'' |title=IDH1 and IDH2 mutations in gliomas |journal=N. Engl. J. Med. |volume=360 |issue=8 |pages=765–73 |year=2009 |month=February |pmid=19228619 |pmc=2820383 |doi=10.1056/NEJMoa0808710 |url=}}</ref><ref name=pmid20975057>{{cite journal |author=Houillier C, Wang X, Kaloshi G, ''et al.'' |title=IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas |journal=Neurology |volume=75 |issue=17 |pages=1560–6 |year=2010 |month=October |pmid=20975057 |doi=10.1212/WNL.0b013e3181f96282 |url=}}</ref>
*[[H3F3A|H3F3A K27M]] in [[Diffuse midline glioma, H3 K27-altered]].
*[[ATRX]] -ve in [[Astrocytoma, IDH-mutant]] or [[Diffuse hemispheric glioma, H3 G34-mutant]].
*[[CD20]] in PCNSL.
*Cytokeratins in Carcinoma brain metastases, Plexus choroid tumours, [[AT/RT]], [[Papillary tumour of the pineal region]], [[Craniopharyngioma]].
*[[EMA]] in [[Meningioma]] and carcinoma brain metastases.
*PrgR in [[Meningioma]] and carcinoma metastases.
*[[Synaptophysin]] in glioneuronal tumours and Pituitary adenoma and embryonal tumours.


===Common neuropathology tumours in a table===
===Common neuropathology tumours in a table===
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|variable
|variable
|missed lesion / close to a lesion; non-specific pathologic process - need more tissue
|missed lesion / close to a lesion; non-specific pathologic process - need more tissue
|nil
|GFAP
|[[Image:Reactive_astrocytes_-_lfb_-_high_mag.jpg|thumb|center|150px|Reactive astrocytes. (WC)]]
|[[Image:Reactive_astrocytes_-_lfb_-_high_mag.jpg|thumb|center|150px|Reactive astrocytes. (WC)]]
|-
|-
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|old or young
|old or young
|need frozen section to Dx, DDx: [[meningioma]]
|need frozen section to Dx, DDx: [[meningioma]]
|S100
|S100, SOX10
|[[Image:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg|thumb|center|150px|Schwannoma. (WC)]]
|[[Image:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg|thumb|center|150px|Schwannoma. (WC)]]
|-
|-
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|[[Image:Meningioma_intermed_mag.jpg |thumb|center|150px|Meningioma. (WC)]]
|[[Image:Meningioma_intermed_mag.jpg |thumb|center|150px|Meningioma. (WC)]]
|-
|-
|Infiltrative [[astrocytoma]] ([[WHO]] grade II or grade III)
|[[Astrocytoma, IDH-mutant]] (CNS [[WHO]] grade 2 or grade 3)
|glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, no microvascular proliferation, no necrosis
|glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, no microvascular proliferation, no necrosis
|often enhancing (suggests high grade), usu. supratentorial, usu. white matter
|often enhancing (suggests high grade), usu. supratentorial, usu. white matter
|usu. old, occ. young
|usu. old, occ. young
|common
|common
|IDH-1+/-, GFAP+
|IDH-1(R132H)+/-, GFAP+
| [[Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | thumb| center| 150px|High-grade astrocytoma. (WC)]]
| [[Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | thumb| center| 150px|High-grade astrocytoma. (WC)]]
|-
|-
|[[Glioblastoma]] (WHO grade IV)
|[[Glioblastoma]], IDH-wildtype (CNS [[WHO]] grade 4)
|glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, microvascular proliferation or necrosis
|glial processes (esp. on smear), nuclear atypia (typical size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †, microvascular proliferation or necrosis
|often enhancing (suggests high grade), usu. supratentorial, usu. white matter
|often enhancing (suggests high grade), usu. supratentorial, usu. white matter
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{{Main|Brain metastasis}}
{{Main|Brain metastasis}}


==Infiltrative astrocytomas==  
 
===Molecular===
See also:  [[Molecular_pathology_tests#Neuropathology|Molecular Neuropathology]]
 
==Gliomas==
{{Main|Glioma}}
 
Gliomas, glioneuronal tumours and neuronal tumours are often categorized together.
 
===Astrocytic tumours===
{{Main|Astrocytoma}}
{{Main|Astrocytoma}}


===Overview===
* [[Astrocytoma]], IDH-mutant.
*Low-grade (diffuse) astrocytomas (WHO Grade II).
* [[Glioblastoma]], IDH-wildtype.
*Anaplastic astrocytomas (WHO Grade III).
** [[Gliosarcoma]] (a glioblastoma subtype)
*[[Glioblastoma]](WHO Grade IV).
**[[Gliosarcoma]] (WHO Grade IV).
*[[Gliomatosis cerebri]] (Grade III/IV).
 
Notes:
*Non-infiltrative astrocytomas:
**[[Pilocytic astrocytoma]] (WHO Grade I).
***[[Pilomyxoid astrocytoma]] (WHO Grade II).
**[[Pleomorphic xanthoastrocytoma]] (WHO grade II).
**[[Subependymal giant cell astrocytoma]] (WHO grade I).


===Microscopic===
Features:<ref name=pmid>{{cite journal |author=Rong Y, Durden DL, Van Meir EG, Brat DJ |title='Pseudopalisading' necrosis in glioblastoma: a familiar morphologic feature that links vascular pathology, hypoxia, and angiogenesis |journal=J. Neuropathol. Exp. Neurol. |volume=65 |issue=6 |pages=529–39 |year=2006 |month=June |pmid=16783163 |doi= |url=}}</ref><ref>[http://dictionary.reference.com/browse/palisading http://dictionary.reference.com/browse/palisading]</ref>
Features:<ref name=pmid>{{cite journal |author=Rong Y, Durden DL, Van Meir EG, Brat DJ |title='Pseudopalisading' necrosis in glioblastoma: a familiar morphologic feature that links vascular pathology, hypoxia, and angiogenesis |journal=J. Neuropathol. Exp. Neurol. |volume=65 |issue=6 |pages=529–39 |year=2006 |month=June |pmid=16783163 |doi= |url=}}</ref><ref>[http://dictionary.reference.com/browse/palisading http://dictionary.reference.com/browse/palisading]</ref>
*Glial processes - '''key feature'''.
*Glial processes - '''key feature'''.
Line 223: Line 276:
*[http://path.upmc.edu/cases/case368.html Gemistocytic astrocytoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case368.html Gemistocytic astrocytoma - several images (upmc.edu)].


Notes:
Depreceated:
*Glial vs. non-glial tumours:
* Diffuse [[Astrocytoma]]
**Glial: "blends into brain"/gradual transition to non-tumour brain.
* [[Anaplastic astrocytoma]]
**Non-glial: no glial processes.
* [[Gliomatosis cerebri]]
*Rosenthal fibres within the tumour... make it into a [[pilocytic astrocytoma]].
* Spongioblastoma
**Rosenthal fibres may be seen around a (very) slow growing tumour and represent a reactive process.
 
*Inflammatory cells and macrophages should prompt consideration of an alternate diagnosis (e.g. [[cerebral infarct]], [[multiple sclerosis]]) - esp. if this is a primary lesion.<ref>URL: [http://path.upmc.edu/cases/case79/dx.html http://path.upmc.edu/cases/case79/dx.html]. Accessed on: 2 January 2012.</ref>
===Oligodendroglial tumours===
* [[Oligodendroglioma]], IDH-mutant and 1p/19q codeleted.


====Grading====
Depreceated:
Nuclear pleomorphism present:
* Anaplastic oligodendroglioma
*At least grade II (diffuse astrocytoma).
* [[Oligoastrocytoma]]
* Anaplastic oligoastrocytoma


Mitotic figures present:
===Pediatric-type diffuse high-grade glioma===
*At least grade III (anaplastic astrocytoma).
{{Main|Pediatric-type diffuse high-grade glioma}}
* [[Astrocytoma#Diffuse_midline_glioma.2C_H3_K27M_mutant|Diffuse midline glioma H3 K27-mutant]]


Microvascular proliferation ''or'' necrosis with pseudopalisading tumour cells:
===Pediatric-type diffuse low-grade glioma===
*Grade IV (glioblastoma [[AKA]] glioblastoma multiforme).
{{Main|Pediatric-type diffuse low-grade glioma}}


Notes:
*Pseudopalisading tumour cells = high tumour cell density adjacent to regions of necrosis; palisade = a fence of poles forming a defensive barrier or fortification.


====Images====
===Circumscribed astrocytic gliomas===
Glioblastoma:
* [[Pilocytic astrocytoma]] (PA)
<gallery>
** [[Pilomyxoid astrocytoma]] (PMA)
Image:Glioblastoma_%281%29.jpg | Glioblastoma - pseudopalisading of tumour cells (WC)
* [[Pleomorphic xanthoastrocytoma]] (PXA)
Image:Glioblastoma_-_high_mag.jpg | Glioblastoma with fragment of near-normal white matter - high mag. (WC)
* [[Subependymal giant cell astrocytoma]] (SEGA)
</gallery>
* [[Neuropathology_tumours#Astroblastoma|Astroblastoma MN1-altered]].
Anaplastic astrocytoma:
* [[Neuropathology_tumours#Chordoid glioma of the third ventricl|Chordoid glioma]].
<gallery>
Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | Anaplastic astrocytoma - very high mag. (WC)
Image:Anaplastic_astrocytoma_-_gfap_-_very_high_mag.jpg | Anaplastic astrocytoma - GFAP - very high mag. (WC)
</gallery>


=====Table of common gliomas - grading=====
====Astroblastoma====
Histomorphologic comparison of common gliomas:
*No WHO grade yet.<ref>{{Ref WHOCNS|88}}</ref>
{| class="wikitable"
*Very rare superficial tumor of young age.<ref>{{Cite journal  | last1 = Narayan | first1 = S. | last2 = Kapoor | first2 = A. | last3 = Singhal | first3 = MK. | last4 = Jakhar | first4 = SL. | last5 = Bagri | first5 = PK. | last6 = Rajput | first6 = PS. | last7 = Kumar | first7 = HS. | title = Astroblastoma of cerebrum: A rare case report and review of literature. | journal = J Cancer Res Ther | volume = 11 | issue = 3 | pages = 667 | month =  | year =  | doi = 10.4103/0973-1482.140800 | PMID = 26458709 }}</ref>
|'''Entity''' || '''Rosenthal <br>fibres / EGBs''' ||'''Nuclear atypia''' ||'''Mitoses''' || '''Necrosis or MVP''' || '''Infiltrative''' || '''Image'''
*Large, cystic. Pushing margin towards CNS.
|-
*Vasocentric growth, plump cells with absence of fibrillary pattern.
|Pilocytic astrocytoma || yes || usu. no || usu. no || usu. no || no || [[Image:Rosenthal_HE_40x.jpg|thumb|150px]]
*GFAP+ve, Synaptohysin-ve, Olig-2-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.
|-
*Molecular profile overlaps with classical [[CNS-PNET]].
|Low-grade astrocytoma || no || yes || no || no || yes || [[File:Astrocytoma_whoII_HE.jpg|thumb|150px]]
**Gene fusions invoving meningioma gene (MN1)<ref>{{Cite journal | last1 = Sturm | first1 = D. | last2 = Orr | first2 = BA. | last3 = Toprak | first3 = UH. | last4 = Hovestadt | first4 = V. | last5 = Jones | first5 = DT. | last6 = Capper | first6 = D. | last7 = Sill | first7 = M. | last8 = Buchhalter | first8 = I. | last9 = Northcott | first9 = PA. | title = New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs. | journal = Cell | volume = 164 | issue = 5 | pages = 1060-72 | month = Feb | year = 2016 | doi = 10.1016/j.cell.2016.01.015 | PMID = 26919435 }}</ref>
|-
|Anaplastic astrocytoma || no || yes || yes || no || yes || [[Image:Anaplastic_astrocytoma_-_high_mag.jpg|thumb|150px]]
|-
|Glioblastoma || no || yes || yes || yes || yes || [[Image:Glioblastoma_-_high_mag.jpg|thumb|150px]]
|}
Notes:
*''MVP'' = microvascular proliferation.
*''EGBs'' = eosinophilic granular bodies.


===IHC===
*GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane).
*Ki-67 - usu. high >20% of cells.
*p53 - often +ve.
*IDH1 (isocitrate dehydrogenase 1).
**+ve in tumours that arose from low-grade gliomas.<ref name=pmid19228619>{{cite journal |author=Yan H, Parsons DW, Jin G, ''et al.'' |title=IDH1 and IDH2 mutations in gliomas |journal=N. Engl. J. Med. |volume=360 |issue=8 |pages=765–73 |year=2009 |month=February |pmid=19228619 |pmc=2820383 |doi=10.1056/NEJMoa0808710 |url=}}</ref>
***Image: [http://en.wikipedia.org/wiki/File:IDH1_GBM_20x.jpg IDH1 +ve in glioblastoma (WP)].


Notes:
<gallery>
*IDH1 and IDH2 mutations - better survival.<ref name=pmid20975057>{{cite journal |author=Houillier C, Wang X, Kaloshi G, ''et al.'' |title=IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas |journal=Neurology |volume=75 |issue=17 |pages=1560–6 |year=2010 |month=October |pmid=20975057 |doi=10.1212/WNL.0b013e3181f96282 |url=}}</ref>
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
File:Astroblastoma.jpg | Astroblastoma (AFIP)
</gallery>


==Astrocytic tumours==
====Chordoid glioma of the third ventricle====
{{Main|Astrocytoma}}
* WHO grade II.
* Slowly growing, non-invasive, in adults.
* Clusters of epithelioid cells in mucinous stroma.
* Lymphocytic infiltrates, adjacent Rosenthal fibers.
* Fibrosis may be present.
* Few mitoses.
* [[GFAP]]+ve,  MIB-1 1-3%.
* [[TTF-1]]+ve.
* CD34+ve.
* [[IDH-1]]-ve, [[p53]]-ve.
* PRKCA D463H mutations.<ref>{{Cite journal  | last1 = Goode | first1 = B. | last2 = Mondal | first2 = G. | last3 = Hyun | first3 = M. | last4 = Ruiz | first4 = DG. | last5 = Lin | first5 = YH. | last6 = Van Ziffle | first6 = J. | last7 = Joseph | first7 = NM. | last8 = Onodera | first8 = C. | last9 = Talevich | first9 = E. | title = A recurrent kinase domain mutation in PRKCA defines chordoid glioma of the third ventricle. | journal = Nat Commun | volume = 9 | issue = 1 | pages = 810 | month = 02 | year = 2018 | doi = 10.1038/s41467-018-02826-8 | PMID = 29476136 }}</ref>


* Diffuse [[Astrocytoma]]
<gallery>
* [[Anaplastic astrocytoma]]
File:NP op 20201028 009.jpg | Chordoid Glioma. (WC/jensflorian)
* [[Glioblastoma]]
</gallery>
* [[Gliomatosis cerebri]]
* [[Pilocytic astrocytoma]] (PA)
* [[Pilomyxoid astrocytoma]] (PMA)
* [[Pleomorphic xanthoastrocytoma]] (PXA)
* [[Subependymal giant cell astrocytoma]] (SEGA)


==Oligodendroglial tumours==
* [[Oligodendroglioma]]
* Anaplastic oligodendroglioma
* [[Oligoastrocytoma]]
* Anaplastic oligoastrocytoma


==Ependymal tumours==
===Ependymal tumours===
* [[Subependymoma]]
* [[Subependymoma]]
* [[Myxopapillary Ependymoma]]
* [[Myxopapillary Ependymoma]]
Line 314: Line 352:


==Other neuroepithelial tumours==
==Other neuroepithelial tumours==
* [[Neuropathology_tumours#Astroblastoma|Astroblastoma]].
* [[Neuropathology_tumours#Chordoid glioma of the third ventricl|Chordoid glioma of the third ventricle]].
* [[Angiocentric glioma]].
* [[Neuropathology_tumours#Cribiform_neuroepithelial_tumour|Cribifiorm neuroepithelial tumour]].
* [[Neuropathology_tumours#Cribiform_neuroepithelial_tumour|Cribifiorm neuroepithelial tumour]].


===Astroblastoma===
*No WHO grade yet.<ref>{{Ref WHOCNS|88}}</ref>
*Very rare superficial tumor of young age.<ref>{{Cite journal  | last1 = Narayan | first1 = S. | last2 = Kapoor | first2 = A. | last3 = Singhal | first3 = MK. | last4 = Jakhar | first4 = SL. | last5 = Bagri | first5 = PK. | last6 = Rajput | first6 = PS. | last7 = Kumar | first7 = HS. | title = Astroblastoma of cerebrum: A rare case report and review of literature. | journal = J Cancer Res Ther | volume = 11 | issue = 3 | pages = 667 | month =  | year =  | doi = 10.4103/0973-1482.140800 | PMID = 26458709 }}</ref>
*Large, cystic. Pushing margin towards CNS.
*Vasocentric growth, plump cells with absence of fibrillary pattern.
*GFAP+ve, Synaptohysin-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.
<gallery>
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
File:Astroblastoma.jpg | Astroblastoma (AFIP)
</gallery>
===Chordoid glioma of the third ventricle===
* WHO grade II.
* Slowly growing, non-invasive.
* Clusters of epithelioid cells in mucinous stroma.
* Lymphocytic infiltrates, adjacent Rosenthal fibers.
* Few mitoses.
* GFAP+ve,  MIB-1 1-3%.


===Cribiform neuroepithelial tumour===
===Cribiform neuroepithelial tumour===
Line 352: Line 367:


==Neuronal and mixed neuronal/glial tumours==
==Neuronal and mixed neuronal/glial tumours==
* Desmoplastic infantile astrocytoma / ganglioglioma (DIA/DIG)
* [[Desmoplastic infantile astrocytoma]] / ganglioglioma (DIA/DIG)
* [[Dysembryoplastic neuroepithelial tumour]]
* [[Dysembryoplastic neuroepithelial tumour]]
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]]
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]]
* Cerebellar liponeurocytoma
* Cerebellar liponeurocytoma
* [[Papillary glioneuronal tumour]]
* [[Papillary glioneuronal tumour]] (PGNT)
* Rosette-forming glioneuronal tumour of the fourth ventricle
* [[Rosette-forming glioneuronal tumour of the fourth ventricle]] (RGNT)
* Gangliocytoma / Ganglioglioma
* Gangliocytoma / Ganglioglioma
* Dysplastic ganglioglioma of the cerebellum (Lhermitte-Duclos)
* Dysplastic ganglioglioma of the cerebellum ([[Lhermitte-Duclos disease]])
* [[Paraganglioma]]
* [[Paraganglioma]]
===Desmoplastic infantile astrocytoma / Desmoplastic infantile ganglioglioma===
* Abbreviated ''DIA'' or ''DIG''.
* ICD-O code: 9412/1
* Large, superficial, cystic tumor of the infancy.
* Biologic course corresponds to WHO grade I.
* Very rare, included in the WHO since 1993.
* Prominent desmoplastic stroma.
* Astrocytic cells within stroma.
**GFAP+.
**MIB-1 usu. 1%.
* Frequent BRAF V600E or V600D mutations.<ref>{{Cite journal  | last1 = Wang | first1 = AC. | last2 = Jones | first2 = DTW. | last3 = Abecassis | first3 = IJ. | last4 = Cole | first4 = BL. | last5 = Leary | first5 = SES. | last6 = Lockwood | first6 = CM. | last7 = Chavez | first7 = L. | last8 = Capper | first8 = D. | last9 = Korshunov | first9 = A. | title = Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIG/DIA) are Distinct Entities with Frequent BRAFV600 Mutations. | journal = Mol Cancer Res | volume =  | issue =  | pages =  | month = Jul | year = 2018 | doi = 10.1158/1541-7786.MCR-17-0507 | PMID = 30006355 }}</ref>
*Single case with BRAF indel or BRAF fusion.
<gallery>
File:DIG-histology.jpg | Histopathology of DIG (HE stain)
File:DIG-histology2.jpg | Prominent ganglioid cells in DIG (HE stain)
</gallery>


===Cerebellar liponeurocytoma===
===Cerebellar liponeurocytoma===
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* NeuN +ve.
* NeuN +ve.
* MIB-1: usu 1-3%.
* MIB-1: usu 1-3%.
====Molecular====
* Distinct methylation profile.
* Recurent losses on 2p and Chr. 14.<ref>{{Cite journal  | last1 = Capper | first1 = D. | last2 = Stichel | first2 = D. | last3 = Sahm | first3 = F. | last4 = Jones | first4 = DTW. | last5 = Schrimpf | first5 = D. | last6 = Sill | first6 = M. | last7 = Schmid | first7 = S. | last8 = Hovestadt | first8 = V. | last9 = Reuss | first9 = DE. | title = Practical implementation of DNA methylation and copy-number-based CNS tumor diagnostics: the Heidelberg experience. | journal = Acta Neuropathol | volume =  | issue =  | pages =  | month = Jul | year = 2018 | doi = 10.1007/s00401-018-1879-y | PMID = 29967940 }}</ref>


<gallery>
<gallery>
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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).
*ICD-O code: 9505/1 (Anaplastic ganglioglioma: 9505/3)
*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===
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*[[AKA]] ''dysplastic gangliocytoma of the cerebellum''.
*[[AKA]] ''dysplastic gangliocytoma of the cerebellum''.
{{Main|Lhermitte-Duclos disease}}
{{Main|Lhermitte-Duclos disease}}
<gallery>
File:Dysplastic_gangliocytoma_lhermitte_duclos.jpg | Dysplastic gangliocytoma (low mag).
</gallery>


===Papillary glioneuronal tumor===
===Papillary glioneuronal tumour===
* Abbreviated ''PGNT''.
* Abbreviated ''PGNT''.
* A benign, supratentorial tumor of childhood.
* A benign, supratentorial tumor of childhood.
** Biologic course corresponds to WHO I
** Biologic course corresponds to WHO grade I.
** Before WHO 2000, considered a [[Ganglioglioma]] variant.
** Before WHO 2000, considered a [[Ganglioglioma]] variant.
*Prominent pseudopapillary architecture.
*Prominent pseudopapillary architecture.
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File:PGNT_HE_stain.jpg | PGNT (HE) (WC/jensflorian)
File:PGNT_HE_stain.jpg | PGNT (HE) (WC/jensflorian)
</gallery>
</gallery>
===Rosette-forming glioneuronal tumour of the fourth ventricle===
* Abbreviated ''RGNT''.
* Provisional ICD-O code: 9509/1
* A rare benign infratentorial tumour of the midline of children and adults.
* Biologic course corresponds to WHO grade I.
* Glial component corresponds to [[pilocytic astrocytoma]].
* Neurocytic rosettes.
* Eosinopil fibrillary cores / pseudorosettes.
* GFAP+ in fibrillary areas, Syn+ in rosettes.
* Neurocytic cells: MAP2+
* MIB-1 usu. below 3%.
<gallery>
File:Histology RGNT HE.jpg | RGNT, HE stain (WC/jensflorian).
File:RGNT HE 2.jpg | RGNT, higher magnification (WC/jensflorian).
</gallery>
===Polymorphous low-grade tumor of the young (PLNTY)===
* [[Pediatric-type diffuse low-grade glioma#Diffuse low-grade glioma, MAPK pathway-altered|Polymorphous low-grade tumor of the young (PLNTY)]]


==Pineal tumours==
==Pineal tumours==
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* [[Primitive neuroectodermal tumour]] (PNET)
* [[Primitive neuroectodermal tumour]] (PNET)
* [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR)
* [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR)
DDx:
* [[Ewing sarcoma]]
* [[Sarcoma with CIC-rearrangement]]


==Peripheral nerve sheath tumours==
==Peripheral nerve sheath tumours==
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Common pattern:
Common pattern:
*CD20 +ve - key stain.
*[[CD20]] +ve - key stain.
*CD3 -ve.
*CD3 -ve.
*Ki-67 ~40%.
*Ki-67 ~40%.
Jensflorian
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