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==Brain tumours - overview==
==Brain tumours - overview==
===Adult===
===Alphabetical===
For overview see [[:Category:Neuropathology_tumours|here]]
 
===By age group===
====Adult====
Four most common types of brain tumours:<ref>[http://neurosurgery.mgh.harvard.edu/abta/primer.htm http://neurosurgery.mgh.harvard.edu/abta/primer.htm]</ref>
Four most common types of brain tumours:<ref>[http://neurosurgery.mgh.harvard.edu/abta/primer.htm http://neurosurgery.mgh.harvard.edu/abta/primer.htm]</ref>
# Metastatic brain tumours (barely edges out primary tumours)
# Metastatic brain tumours (barely edges out primary tumours)
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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]].


===Children===
====Children====
# Astrocytoma.
# [[Pilocytic astrocytoma]].
# [[Medulloblastoma]].
# [[Medulloblastoma]].
# [[Ependymoma]].
# [[Ependymoma]].
# Pontine glioma, often [[Diffuse midline glioma, H3 K27-altered]].


===Location (most common)===
===By location===
Certain tumours like to hang-out at certain places:<ref>URL: [http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif] and [http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html]. Accessed on: 2 November 2010.</ref>
Certain tumours like to hang-out at certain places:<ref>URL: [http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif] and [http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html]. Accessed on: 2 November 2010.</ref>
*Cerebrum:
====Cerebrum====
**Cortical based - [[oligodendroglioma]].
*Cortical based - [[oligodendroglioma]].
**Grey-white junction - metastases.
*Grey-white junction - metastases.
**White matter - astrocytoma, [[glioblastoma]].
*White matter - astrocytoma, [[glioblastoma]].
**Periventricular - CNS lymphoma.
*Periventricular - CNS lymphoma.
**Cystic - [[ganglioglioma]], [[pilocytic astrocytoma]], [[pleomorphic xanthoastrocytoma]].
*Cystic - [[ganglioglioma]], [[pilocytic astrocytoma]], [[pleomorphic xanthoastrocytoma]].
*Cerebellum:
====Cerebellum====
**Midline/central - [[medulloblastoma]].
*Midline/central - [[medulloblastoma]].
**Cystic lesion - pilocytic astrocytoma (younger individual), [[hemangioblastoma]] (older individual).
*Cystic lesion - pilocytic astrocytoma (younger individual), [[hemangioblastoma]] (older individual).
**Solid lesion (older individual) - [[metastasis]].
*Solid lesion (older individual) - [[metastasis]].
*Spinal cord:
====Sella turcica====
**[[Ependymoma]], glioblastoma.
* [[Pituitary adenoma]].
**Filum terminale - [[myxopapillary ependymoma]], [[paraganglioma]].
* [[Craniopharyngioma]].
 
less common:
* [[Pituicytoma]].
* [[Granular cell tumour]].
* [[Germinoma]].
* [[Chordoma]]
* Rathke cleft cyst.
* Hypophysitis.
* Xanthogranuloma.
====Spinal cord====
*[[Ependymoma]]
*[[Glioblastoma]]
*[[Meningioma]]
*Carcinoma metastasis
*[[Hemangioblastoma]]
====Filum terminale====
====Filum terminale====
*Filum terminale = bottom end of the spinal cord - has a limited differential.
DDx:<ref>JLK. 31 May 2010.</ref>
*[[Meningioma]].
*[[Meningioma]].
*[[Myxopapillary ependymoma]].
*[[Myxopapillary ependymoma]].
Line 46: Line 61:
*[[Schwannoma]].
*[[Schwannoma]].
*[[Paraganglioma]].
*[[Paraganglioma]].
 
====Meninges====
====Cerebellopontine angle====
* [[Meningioma]].
*Abbreviated ''CP angle''.
* [[Solitary fibrous tumour]] / Hemangiopericytoma.
 
* [[Hemangioblastoma]].
DDx:<ref>R. Kiehl. 8 November 2010.</ref>
less common:
*[[Schwannoma]].
* [[Melanoma]] / Melanocytoma.
*[[Meningioma]].
* Lymphoproliferative diseases.
*[[Dermoid cyst]]/epidermoid cyst.
* [[Sarcoidosis]]
*[[Ependymoma]].
* [[Arachnoid cyst]].
*[[Choroid plexus papilloma]].
* Disseminated oligodendroglial-like leptomeningeal tumour.
 
* Desmoplastic infantile astrocytoma / ganglioglioma.
===Cystic tumours===
* Meningioangiomatosis.
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>
* Calcifying pseudoneoplasm.
*[[Pilocytic astrocytoma]].  
====Skull====
*[[Pleomorphic xanthoastrocytoma]].  
* [[Fibrous dysplasia]].
*[[Ganglioglioma]].
* [[Paget disease]].
*[[Hemangioblastoma]].
* [[Histiocytosis]].
*[[Craniopharyngioma]].<ref>URL: [http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral]. Accessed on: 14 January 2012.</ref>
* [[Hemangioma]].
* [[Aneurysmal bone cyst]].
* [[Plasma_cell_neoplasms#Multiple_myeloma|Multiple myeloma]].
====Skull base / Cerebellopontine angle====
* [[Schwannoma]].
* [[Meningioma]].
* [[Dermoid cyst]] / epidermoid cyst.
less common:
* [[Ependymoma]].
* [[Choroid plexus papilloma]].
* [[Glomus tumour]].
* [[Chordoma]].
* [[Chondrosarcoma]].
* [[Olfactory neuroblastoma]].
* [[Endolymphatic sac tumour]].


===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)]]
|-
|-
|[[Astrocytoma]] (grade II or worse)
|[[Schwannoma]]
|glial processes (esp. on smear), nuclear atypia (size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †
|cellular areas (Antoni A), paucicelluar areas (Antoni B), palisading of nuclei (Verocay bodies)
|extra-axial + intradural
|old or young
|need frozen section to Dx, DDx: [[meningioma]]
|S100, SOX10
|[[Image:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg|thumb|center|150px|Schwannoma. (WC)]]
|-
|[[Meningioma]]
|whorls, psammomatous calcs, nuclear inclusions
|extra-axial + intradural
|old or young
|may be diagnosed on smear, DDx: [[schwannoma]], choroid plexus
|EMA, PR, Ki-67
|[[Image:Meningioma_intermed_mag.jpg |thumb|center|150px|Meningioma. (WC)]]
|-
|[[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
|often enhancing (suggests high grade), usu. supratentorial, usu. white matter
|usu. old, occ. young
|common
|IDH-1(R132H)+/-, GFAP+
| [[Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | thumb| center| 150px|High-grade astrocytoma. (WC)]]
|-
|[[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
|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
|very common, esp. glioblastoma
|very common, esp. glioblastoma
|IDH-1+/-, GFAP+
|IDH-1+/-, GFAP+
| [[Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | thumb| center| 150px|Astrocytoma. (WC)]]
| [[Image:Glioblastoma (1).jpg | thumb| center| 150px|Glioblastoma. (WC)]]
|-
|-
|[[Metastatic brain tumours|Metastasis]]
|[[Metastatic brain tumours|Metastasis]]
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|[[TTF-1]], CK7, [[CK20]], BRST-2
|[[TTF-1]], CK7, [[CK20]], BRST-2
|[[Image:Metastatic_adenocarcinoma_-_cerebellum_-_very_low_mag.jpg | thumb| center|150px |Metastasis. (WC)]]
|[[Image:Metastatic_adenocarcinoma_-_cerebellum_-_very_low_mag.jpg | thumb| center|150px |Metastasis. (WC)]]
|-
|[[Meningioma]]
|whorls, psammomatous calcs, nuclear inclusions
|extra-axial + intradural
|old or young
|may be diagnosed on smear, DDx: [[schwannoma]], choroid plexus
|EMA, PR, Ki-67
|[[Image:Meningioma_intermed_mag.jpg |thumb|center|150px|Meningioma. (WC)]]
|-
|[[Schwannoma]]
|cellular areas (Antoni A), paucicelluar areas (Antoni B), palisading of nuclei (Verocay bodies)
|extra-axial + intradural
|old or young
|need frozen section to Dx, DDx: [[meningioma]]
|S100
|[[Image:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg|thumb|center|150px|Schwannoma. (WC)]]
|}
|}
† Rosenthal fibres at the periphery of a lesion are a non-specific finding seen in chronic processes.
† Rosenthal fibres at the periphery of a lesion are a non-specific finding seen in chronic processes.
Line 151: Line 250:
{{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 (Grade II).
* [[Glioblastoma]], IDH-wildtype.
*Anaplastic astrocytomas (Grade III).
** [[Gliosarcoma]] (a glioblastoma subtype)
*Glioblastoma (Grade IV).


Notes:
*Non-infiltrative gliomas:
**[[Pilocytic astrocytoma]] (WHO Grade I).
**[[Dysembryoplastic neuroepithelial tumour]] (DNT), (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 175: 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>


====Grading====
===Oligodendroglial tumours===
Nuclear pleomorphism present:
* [[Oligodendroglioma]], IDH-mutant and 1p/19q codeleted.
*At least grade II (diffuse astrocytoma).


Mitotic figures present:
Depreceated:
*At least grade III (anaplastic astrocytoma).
* Anaplastic oligodendroglioma
* [[Oligoastrocytoma]]
* Anaplastic oligoastrocytoma


Microvascular proliferation ''or'' necrosis with pseudopalisading tumour cells:
===Pediatric-type diffuse high-grade glioma===
*Grade IV (glioblastoma [[AKA]] glioblastoma multiforme).
{{Main|Pediatric-type diffuse high-grade glioma}}
* [[Astrocytoma#Diffuse_midline_glioma.2C_H3_K27M_mutant|Diffuse midline glioma H3 K27-mutant]]


Notes:
===Pediatric-type diffuse low-grade glioma===
*Pseudopalisading tumour cells = high tumour cell density adjacent to regions of necrosis; palisade = a fence of poles forming a defensive barrier or fortification.
{{Main|Pediatric-type diffuse low-grade glioma}}


Images:
*Glioblastoma:
**[http://commons.wikimedia.org/wiki/File:Glioblastoma_%281%29.jpg Glioblastoma - pseudopalisading of tumour cells (WC)].
**[http://commons.wikimedia.org/wiki/File:Glioblastoma_-_high_mag.jpg Glioblastoma with fragment of near-normal white matter - high mag. (WC)].
*Anaplastic astrocytoma:
**[http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg Anaplastic astrocytoma - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma_-_gfap_-_very_high_mag.jpg Anaplastic astrocytoma - GFAP - very high mag. (WC)].


=====Table of common gliomas - grading=====
===Circumscribed astrocytic gliomas===
Histomorphologic comparison of common gliomas:
* [[Pilocytic astrocytoma]] (PA)
{| class="wikitable"
** [[Pilomyxoid astrocytoma]] (PMA)
|'''Entity''' || '''Rosenthal <br>fibres / EGBs''' ||'''Nuclear atypia''' ||'''Mitoses''' || '''Necrosis or MVP''' || '''Infiltrative''' || '''Image'''
* [[Pleomorphic xanthoastrocytoma]] (PXA)
|-
* [[Subependymal giant cell astrocytoma]] (SEGA)
|Pilocytic astrocytoma || yes || usu. no || usu. no || usu. no || no || [http://commons.wikimedia.org/wiki/File:Rosenthal_HE_40x.jpg]
* [[Neuropathology_tumours#Astroblastoma|Astroblastoma MN1-altered]].
|-
* [[Neuropathology_tumours#Chordoid glioma of the third ventricl|Chordoid glioma]].
|Low-grade astrocytoma || no || yes || no || no || yes || image?
|-
|Anaplastic astrocytoma || no || yes || yes || no || yes || [http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma_-_high_mag.jpg]
|-
|Glioblastoma || no || yes || yes || yes || yes || [http://commons.wikimedia.org/wiki/File:Glioblastoma_-_high_mag.jpg]
|}
Notes:
*''MVP'' = microvascular proliferation.
*''EGBs'' = eosinophilic granular bodies.


===IHC===
====Astroblastoma====
*GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane).
*No WHO grade yet.<ref>{{Ref WHOCNS|88}}</ref>
*Ki-67 - usu. high >20% of cells.
*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>
*p53 - often +ve.
*Large, cystic. Pushing margin towards CNS.
*IDH1 (isocitrate dehydrogenase 1).
*Vasocentric growth, plump cells with absence of fibrillary pattern.
**+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>
*GFAP+ve, Synaptohysin-ve, Olig-2-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.
***Image: [http://en.wikipedia.org/wiki/File:IDH1_GBM_20x.jpg IDH1 +ve in glioblastoma (WP)].
*Molecular profile overlaps with classical [[CNS-PNET]].
**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>


Notes:
*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>


==Pilocytic astrocytoma==
<gallery>
{{Main|Pilocytic astrocytoma}}
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
File:Astroblastoma.jpg | Astroblastoma (AFIP)
</gallery>


==Pleomorphic xanthoastrocytoma==
====Chordoid glioma of the third ventricle====
*Abbreviated ''PXA''.
* WHO grade II.
===General===
* Slowly growing, non-invasive, in adults.
Features:
* Clusters of epithelioid cells in mucinous stroma.
*Classically in the temporal lobe in children and young adults.
* Lymphocytic infiltrates, adjacent Rosenthal fibers.
*Associated with seizures.
* Fibrosis may be present.
*Moderately aggressive (WHO Grade II).<ref name=pmid11465399>{{Cite journal  | last1 = Fouladi | first1 = M. | last2 = Jenkins | first2 = J. | last3 = Burger | first3 = P. | last4 = Langston | first4 = J. | last5 = Merchant | first5 = T. | last6 = Heideman | first6 = R. | last7 = Thompson | first7 = S. | last8 = Sanford | first8 = A. | last9 = Kun | first9 = L. | title = Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. | journal = Neuro Oncol | volume = 3 | issue = 3 | pages = 184-92 | month = Jul | year = 2001 | doi = | PMID = 11465399 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1920613/pdf/11465399.pdf}}</ref>
* 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>


===Gross===
<gallery>
*Temporal lobe - classic.
File:NP op 20201028 009.jpg | Chordoid Glioma. (WC/jensflorian)
*Usually assoc. with the leptomeninges,<ref name=pmid11465399/> i.e. superficial.
</gallery>


===Microscopic===
Features:<ref name=Ref_PBoD8_1333>{{Ref PBoD8|1333}}</ref>
*Marked nuclear atypia.
*Eosinophilic granular bodies - very common.<ref name=pmid11465399/>
*Inflammation (chronic).


Notes:
===Ependymal tumours===
*No mitoses.
* [[Subependymoma]]
*No [[necrosis]].
* [[Myxopapillary Ependymoma]]
* [[Ependymoma]]
* Anaplastic ependymoma


Images:
==Choroid plexus tumours==
*[http://path.upmc.edu/cases/case499.html Pleomorphic xanthoastrocytoma - several images (upmc.edu)].
* [[Choroid plexus papilloma]]
*[http://path.upmc.edu/cases/case511.html Pleomorphic xanthoastrocytoma with anaplasia - another case - several images (upmc.edu)].
* Atypical choroid plexus papilloma
*[http://path.upmc.edu/cases/case578.html Pleomorphic xanthoastrocytoma with anaplasia - case 3 - several images (upmc.edu)].
* [[Choroid plexus carcinoma]]
*[http://path.upmc.edu/cases/case579.html Cerebellar pleomorphic xanthoastrocytoma - case 4 - several image (upmc.edu)].


===Stains===
==Other neuroepithelial tumours==
*[[Reticulin stain]] - intercellular, prominent.<ref name=pmid21479234>{{Cite journal  | last1 = Dias-Santagata | first1 = D. | last2 = Lam | first2 = Q. | last3 = Vernovsky | first3 = K. | last4 = Vena | first4 = N. | last5 = Lennerz | first5 = JK. | last6 = Borger | first6 = DR. | last7 = Batchelor | first7 = TT. | last8 = Ligon | first8 = KL. | last9 = Iafrate | first9 = AJ. | title = BRAF V600E mutations are common in pleomorphic xanthoastrocytoma: diagnostic and therapeutic implications. | journal = PLoS One | volume = 6 | issue = 3 | pages = e17948 | month =  | year = 2011 | doi = 10.1371/journal.pone.0017948 | PMID = 21479234 }}</ref>
* [[Neuropathology_tumours#Cribiform_neuroepithelial_tumour|Cribifiorm neuroepithelial tumour]].


Image:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3066220/figure/pone-0017948-g001/ PXA - several images (nih.gov)].<ref name=pmid21479234/>


===IHC===
===Cribiform neuroepithelial tumour===
*GFAP +ve.
AKA: '''CRINET'''.
*CD68 +ve.
*Not listed in the current WHO classification.
*First description in 2009.<ref>{{Cite journal  | last1 = Hasselblatt | first1 = M. | last2 = Oyen | first2 = F. | last3 = Gesk | first3 = S. | last4 = Kordes | first4 = U. | last5 = Wrede | first5 = B. | last6 = Bergmann | first6 = M. | last7 = Schmid | first7 = H. | last8 = Frühwald | first8 = MC. | last9 = Schneppenheim | first9 = R. | title = Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis. | journal = J Neuropathol Exp Neurol | volume = 68 | issue = 12 | pages = 1249-55 | month = Dec | year = 2009 | doi = 10.1097/NEN.0b013e3181c06a51 | PMID = 19915490 }}</ref>
*Around ventricles.<ref>{{Cite journal  | last1 = Arnold | first1 = MA. | last2 = Stallings-Archer | first2 = K. | last3 = Marlin | first3 = E. | last4 = Grondin | first4 = R. | last5 = Olshefski | first5 = R. | last6 = Biegel | first6 = JA. | last7 = Pierson | first7 = CR. | title = Cribriform neuroepithelial tumor arising in the lateral ventricle. | journal = Pediatr Dev Pathol | volume = 16 | issue = 4 | pages = 301-7 | month =  | year =  | doi = 10.2350/12-12-1287-CR.1 | PMID = 23495723 }}</ref>
*Young children.<ref>{{Cite journal  | last1 = Park | first1 = JY. | last2 = Kim | first2 = E. | last3 = Kim | first3 = DW. | last4 = Chang | first4 = HW. | last5 = Kim | first5 = SP. | title = Cribriform neuroepithelial tumor in the third ventricle: a case report and literature review. | journal = Neuropathology | volume = 32 | issue = 5 | pages = 570-6 | month = Oct | year = 2012 | doi = 10.1111/j.1440-1789.2011.01293.x | PMID = 22239490 }}</ref>
*Small undifferentiated cells arranged in cribriform strands and trabeculae of varying thickness.
*MAP2+ve, Synaptophysin+ve, CK+/-ve. MIB-1: 30%.
*INI-1 loss, but no rhabdoid features and good prognosis.  
*Stable genomic profile.<ref>{{Cite journal  | last1 = Gessi | first1 = M. | last2 = Japp | first2 = AS. | last3 = Dreschmann | first3 = V. | last4 = Zur Mühlen | first4 = A. | last5 = Goschzik | first5 = T. | last6 = Dörner | first6 = E. | last7 = Pietsch | first7 = T. | title = High-Resolution Genomic Analysis of Cribriform Neuroepithelial Tumors of the Central Nervous System. | journal = J Neuropathol Exp Neurol | volume = 74 | issue = 10 | pages = 970-4 | month = Oct | year = 2015 | doi = 10.1097/NEN.0000000000000239 | PMID = 26352987 }}</ref>


==Dysembryoplastic neuroepithelial tumour==
==Neuronal and mixed neuronal/glial tumours==
*Abbreviated ''DNT''.
* [[Desmoplastic infantile astrocytoma]] / ganglioglioma (DIA/DIG)
{{Main|Dysembryoplastic neuroepithelial tumour}}
* [[Dysembryoplastic neuroepithelial tumour]]
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]]
* Cerebellar liponeurocytoma
* [[Papillary glioneuronal tumour]] (PGNT)
* [[Rosette-forming glioneuronal tumour of the fourth ventricle]] (RGNT)
* Gangliocytoma / Ganglioglioma
* Dysplastic ganglioglioma of the cerebellum ([[Lhermitte-Duclos disease]])
* [[Paraganglioma]]


==Subependymal giant cell astrocytoma==
===Desmoplastic infantile astrocytoma / Desmoplastic infantile ganglioglioma===
*Abbreviated ''SEGA''.
* Abbreviated ''DIA'' or ''DIG''.
{{Main|Subependymal giant cell astrocytoma}}
* 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>


==Pilomyxoid astrocytoma==
===Cerebellar liponeurocytoma===
{{Main|Pilomyxoid astrocytoma}}
* Previously called ''lipomatous medulloblastoma'' (name changed in WHO 2000).
* Mean age: 50 years.
* As the name states: A tumour of the cerebellum.
** But cases outside cerebellum reported that would qualify.<ref>{{Cite journal  | last1 = Gupta | first1 = K. | last2 = Salunke | first2 = P. | last3 = Kalra | first3 = I. | last4 = Vasishta | first4 = RK. | title = Central liponeurocytoma: case report and review of literature. | journal = Clin Neuropathol | volume = 30 | issue = 2 | pages = 80-5 | month =  | year =  | doi =  | PMID = 21329617 }}</ref>
* WHO grade II <ref>{{Cite journal  | last1 = Nishimoto | first1 = T. | last2 = Kaya | first2 = B. | title = Cerebellar liponeurocytoma. | journal = Arch Pathol Lab Med | volume = 136 | issue = 8 | pages = 965-9 | month = Aug | year = 2012 | doi = 10.5858/arpa.2011-0337-RS | PMID = 22849747 }}</ref> (upgraded from WHO grade I in 2007)<ref>{{Cite journal  | last1 = Brat | first1 = DJ. | last2 = Parisi | first2 = JE. | last3 = Kleinschmidt-DeMasters | first3 = BK. | last4 = Yachnis | first4 = AT. | last5 = Montine | first5 = TJ. | last6 = Boyer | first6 = PJ. | last7 = Powell | first7 = SZ. | last8 = Prayson | first8 = RA. | last9 = McLendon | first9 = RE. | title = Surgical neuropathology update: a review of changes introduced by the WHO classification of tumours of the central nervous system, 4th edition. | journal = Arch Pathol Lab Med | volume = 132 | issue = 6 | pages = 993-1007 | month = Jun | year = 2008 | doi = 10.1043/1543-2165(2008)132[993:SNUARO]2.0.CO;2 | PMID = 18517285 }}</ref>
*ICD-O code: 9506/1


==Atypical teratoid/rhabdoid tumour==
====Histo====
:See also: ''[[Extrarenal malignant rhabdoid tumour]]''.
* Advanced neuronal and lipomatous differentiation.
*Commonly abbreviated ''AT/RT''.
* Neurocytes: round to oval nuclei with clear cytoplasm.
*May be written ''atypical teratoid rhabdoid tumour'', i.e. without the forward slash, or ''atypical teratoid-rhabdoid tumour'' (AT-RT).
* Quite cellular.
{{Main|Atypical teratoid/rhabdoid tumour}}
* Mitoses almost absent.  


==Oligodendroglioma==
====IHC====
{{Main|Oligodendroglioma}}
* [[GFAP]] +/-ve (focal).
* [[MAP2]] +ve.
* Synaptophysin +ve.
* NeuN +ve.
* MIB-1: usu 1-3%.


==Oligoastrocytoma==
====Molecular====
===General===
* Distinct methylation profile.
*Mixed tumour.
* 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>


===Microscopic===
<gallery>
Features:
File:Cerebellar liponeurocytoma.jpg | Liponeurocytoma, HE (WC/Marvin101).
*Astrocytoma-like and oligodendroglioma-like:
File:Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/Marvin101).
*#Oligodendroglioma-like cells = round nucleus, peri-nuclear clearing.
File:Cerebellar Liponeurocytoma HE.jpg | Liponeurocytoma, HE (WC/jensflorian).
*#Astrocytoma-like cells = non-ovoid/elongated nucleus.
File:Cerebellar Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/jensflorian).
</gallery>


DDx:
====DDx====
*Anaplastic astrocytoma.
* [[Medulloblastoma]]
*Oligodendroglioma. (???)
* [[Neurocytoma]]


===IHC===
===Gangliocytoma===
*Oligodendroglioma-like cells: MAP-2 +ve (cytoplasm).
* Grade I WHO neuronal tumour.
*Astrocytoma-like cells: GFAP +ve (cytoplasm, nuclear membrane).
** ICD-O code: 9492/0
* Groups of irregular large neurons.
* Non-neoplastic, reticulin-rich glial stroma.


Others:
===Ganglioglioma===
*Ki-67 ~10%. (???)
:'''Not''' to be confused with ''[[ganglioneuroma]]''.
*p53 - focally +ve. (???)
====General====
*IDH-1 +ve. (85%)
*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]].<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.


==Meningioma==
====Macroscopic====
{{Main|Meningioma}}
*Circumscribed lesion.
===General===
*Usu. contrast enhancing.
*Very common.
*Solid, but intracortical cysts may be present.
*May be part of a syndrome.
*Little mass effect.


===Microscopic===
Features (memory device ''WCN''):
*Whorled appearance - '''key feature'''.
*Calcification, psammomatous.
*[[Nuclear pseudoinclusions]] - focal nuclear clearing with a sharp interface to unremarkable chromatin.


Grading: see ''[[meningioma]]''.
====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.


==Peripheral nerve sheath tumours==
Anaplastic ganglioglioma:
{{Main|Peripheral nerve sheath tumours}}
*Brisk mitotic activity
A classification:<ref name=pmid17893219>{{cite journal |author=Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A |title=Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns |journal=AJNR Am J Neuroradiol |volume=28 |issue=9 |pages=1633–8 |year=2007 |month=October |pmid=17893219 |doi=10.3174/ajnr.A0682 |url=http://www.ajnr.org/cgi/reprint/28/9/1633}}</ref>
*Necrosis
*Benign:
**[[Schwannoma]].
**[[Neurofibroma]].
**[[Perineurioma]].
**[[Traumatic neuroma]].
*Malignant:
**[[Malignant peripheral nerve sheath tumour]] (MPNST).


==Schwannoma==
====IHC====
*Neurons:
**[[MAP2]] +ve
**Synaptophysin +ve
** Neurofilament +ve
*Glia:
**CD34+/-ve
*BRAF V600E +ve (approx. 25%, mainly ganglion cells).


{{Main|Schwannoma}}
====Molecular====
===General===
*BRAF V600E-mutated(approx. 25%).
*Tumour of tissue surrounding a nerve.
*IDH1/2 wt.
**Axons adjacent to the tumour are normal... but may be compressed.
*No 1p/19q codeletion.
*Usu. Chr. 7 gain.
*CDKN2A deletions in anaplastic ganglioglioma.  


===Microscopic===
====DDx:====
Features:<ref name=pmid17893219>{{cite journal |author=Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A |title=Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns |journal=AJNR Am J Neuroradiol |volume=28 |issue=9 |pages=1633–8 |year=2007 |month=October |pmid=17893219 |doi=10.3174/ajnr.A0682 |url=http://www.ajnr.org/cgi/reprint/28/9/1633}}</ref>
*[[DNT]].
*Antoni A:
*[[Oligodendroglioma]].
**Cellular.
*Trapped cortical neurons in diffuse astrocytoma.
**'Fibrillary, polar, elongated'.
*Papillary glioneuronal tumor.
*Antoni B:
*Dysembryoplastic neuroepithelial tumor.
**Pauci-cellular.
**Loose microcystic tissue.
*Verocay bodies - paucinuclear area surrounded by palisaded nuclei.
*In the GI tract: classically have a ''peripheral lymphoid cuff''.<ref name=pmid15728600>{{cite journal |author=Levy AD, Quiles AM, Miettinen M, Sobin LH |title=Gastrointestinal schwannomas: CT features with clinicopathologic correlation |journal=AJR Am J Roentgenol |volume=184 |issue=3 |pages=797–802 |year=2005 |month=March |pmid=15728600 |doi= |url=http://www.ajronline.org/cgi/content/full/184/3/797}}</ref>


Images:
====Images====
*[http://www.pathguy.com/~lulo/lulo0003.htm Antoni A (pathguy.com)].
<gallery>
*[http://www.ajnr.org/cgi/content/full/28/9/1633/F8 Antoni A & Antoni B side-by-side (ajnr.org)].
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing 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:Anaplastic ganglioglioma HE.jpg | Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian)
</gallery>
*[http://path.upmc.edu/cases/case142.html Ganglioglioma - case 1 (upmc.edu)].
*[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)].


Notes:
===Lhermitte-Duclos disease===
*Several subtypes exist.
*Abbreviated ''LDD''.
*[[AKA]] ''dysplastic cerebellar gangliocytoma''.<ref name=pmid20060133>{{Cite journal  | last1 = Yağci-Küpeli | first1 = B. | last2 = Oguz | first2 = KK. | last3 = Bilen | first3 = MA. | last4 = Yalçin | first4 = B. | last5 = Akalan | first5 = N. | last6 = Büyükpamukçu | first6 = M. | title = An unusual cause of posterior fossa mass: Lhermitte-Duclos disease. | journal = J Neurol Sci | volume = 290 | issue = 1-2 | pages = 138-41 | month = Mar | year = 2010 | doi = 10.1016/j.jns.2009.12.010 | PMID = 20060133 }}</ref>
*[[AKA]] ''dysplastic gangliocytoma of the cerebellum''.
{{Main|Lhermitte-Duclos disease}}
<gallery>
File:Dysplastic_gangliocytoma_lhermitte_duclos.jpg | Dysplastic gangliocytoma (low mag).
</gallery>


==Neurofibroma==
===Papillary glioneuronal tumour===
{{Main|Peripheral nerve sheath tumours#Neurofibroma}}
* Abbreviated ''PGNT''.
===General===
* A benign, supratentorial tumor of childhood.
*May be a part of [[neurofibromatosis]] 1.
** Biologic course corresponds to WHO grade I.
*Composed of Schwann cells, axons, fibrous material.<ref name=pmid17893219/>
** Before WHO 2000, considered a [[Ganglioglioma]] variant.
*Prominent pseudopapillary architecture.
*Neurocytes to medium-sized ganglion cells.
*GFAP+ core, GFAP- layer
*Rosenthal fibers, Eosinophilic Granular bodies and lymphocytic cuffing may be present.
<gallery>
File:PGNT_HE_stain.jpg | PGNT (HE) (WC/jensflorian)
</gallery>


===Microscopic===
===Rosette-forming glioneuronal tumour of the fourth ventricle===
Features:
* Abbreviated ''RGNT''.
*Spindle cells lesion.
* Provisional ICD-O code: 9509/1
**See ''[[Neurofibroma]]'' article for details.
* 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>


Image:
===Polymorphous low-grade tumor of the young (PLNTY)===
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(1).jpg Neurofibroma - low mag. (WC)].
* [[Pediatric-type diffuse low-grade glioma#Diffuse low-grade glioma, MAPK pathway-altered|Polymorphous low-grade tumor of the young (PLNTY)]]
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(2).jpg Neurofibroma - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(3).jpg Neurofibroma - high mag. (WC)].


==Ganglioneuroma==
==Pineal tumours==
:'''Not''' to be confused with ''[[ganglioglioma]]''.
{{Main|Pineal gland}}
*[[AKA]] ganglioma.<ref>URL: [http://medical-dictionary.thefreedictionary.com/ganglioma http://medical-dictionary.thefreedictionary.com/ganglioma]. Accessed on: 8 November 2010.</ref>
===General===
*May be retroperitoneal.
*Occasionally found in the GI tract - may form [[Gastrointestinal_tract_polyps#Ganglioneuroma|colonic polyp]].
*Multiple ganglioneuromas may be due to [[multiple endocrine neoplasia IIb]].


Classification:
* [[Pineocytoma]]
*In a grouping known as ''neuroblastic tumours'' which includes:<ref name=pmid10421272>{{cite journal |author=Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B |title=Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee |journal=Cancer |volume=86 |issue=2 |pages=349–63 |year=1999 |month=July |pmid=10421272 |doi= |url=}}</ref>
* [[Pineal parenchymal tumour of intermediate differentiation]]
**Ganglioneuroma (benign).
* [[Pineoblastoma]]
**[[Ganglioneuroblastoma]] (intermediate).
* [[Papillary tumour of the pineal region]]
**[[Neuroblastoma]] (aggressive).


===Gross===
==Embryonal tumours==
*Solid.
* [[Atypical teratoid/rhabdoid tumour]] (AT/RT) or (AT-RT)
*White.
* [[Medulloblastoma]]
*Firm.
* [[Primitive neuroectodermal tumour]] (PNET)
*Well-circumscribed.
* [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR)
*May be nodular.


Images:
DDx:
*[http://commons.wikimedia.org/wiki/File:Adrenal_ganglioneuroma_02.JPG Adrenal ganglioneuroma (WC)].
* [[Ewing sarcoma]]
*[http://www.webpathology.com/image.asp?case=84&n=1 Ganglioneuroma (webpathology.com)].
* [[Sarcoma with CIC-rearrangement]]


===Microscopic===
==Peripheral nerve sheath tumours==
Features:
{{Main|Peripheral nerve sheath tumours}}
*Ganglion cells - '''key feature'''.
A classification:<ref name=pmid17893219>{{cite journal |author=Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A |title=Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns |journal=AJNR Am J Neuroradiol |volume=28 |issue=9 |pages=1633–8 |year=2007 |month=October |pmid=17893219 |doi=10.3174/ajnr.A0682 |url=http://www.ajnr.org/cgi/reprint/28/9/1633}}</ref>
**Large cells with large nucleus.
'''Benign:'''
***Prominent nucleolus.
*[[Schwannoma]].
*Disordered fibrinous-like material.
*[[Neurofibroma]].
*Eosinophilic granular bodies.<ref>R. Kiehl. 8 November 2010.</ref>
*[[Perineurioma]].
*Ganglioneuroma.
**[[Traumatic neuroma]].
'''Malignant:'''
*[[Malignant peripheral nerve sheath tumour]] (MPNST).


Images:
===Ganglioneuroma===
*[http://it.wikipedia.org/wiki/File:Ganglioneuroma_(1).jpg Ganglioneuroma (WC)].
:'''Not''' to be confused with ''[[ganglioglioma]]''.
*[http://www.webpathology.com/image.asp?n=4&Case=84 Ganglioneuroma (webpathology.com)].
*[[AKA]] ganglioma.<ref>URL: [http://medical-dictionary.thefreedictionary.com/ganglioma http://medical-dictionary.thefreedictionary.com/ganglioma]. Accessed on: 8 November 2010.</ref>
*[http://www.webpathology.com/image.asp?case=412&n=4 Ganglioneuroma (webpathology.com)].
{{Main|Ganglioneuroma}}
*[http://en.wikipedia.org/wiki/File:Ganglion_high_mag.jpg Normal ganglion - high mag. (WC)] .


See: ''[[adrenal ganglioneuroma]]'', ''[[Gastrointestinal tract polyps#Ganglioneuroma|colonic ganglioneuroma]]''.


===IHC===
Features:<ref>{{Ref GLP|217}}</ref>
*Spindle cells: S-100 +ve.
*Ganglion cells: NSE, synaptophysin, NF.


==Ependymoma==
==Meningioma==
{{Main|Ependymoma}}
{{Main|Meningioma}}
 
==Subependymoma==
{{Main|Subependymoma}}
 
==Choroid plexus papilloma==
{{Main|Choroid plexus papilloma}}
 
==Choroid plexus carcinoma==
{{Main|Choroid plexus carcinoma}}


==Chordoma==
==Chordoma==
Line 445: Line 596:
==Hemangioblastoma==
==Hemangioblastoma==
{{Main|Hemangioblastoma}}
{{Main|Hemangioblastoma}}
==Medulloblastoma==
{{Main|Medulloblastoma}}
*Tumour of cerebellum - key feature.
*Morphologically identical supratentorial tumours are called [[primitive neuroectodermal tumour]] (PNET).
==Primitive neuroectodermal tumour==
*[[AKA]] ''primitive neuroepithelial tumour''. (???)
===General===
*Abbreviated ''PNET''.
*Should not be confused with ''peripheral primitive neuroectodermal tumour'' (abbreviated ''[[pPNET]]''<ref name=PST14feb11>PST. 14 February 2011.</ref>), [[AKA]] ''[[Ewing sarcoma]]''.
===Microscopic===
Features:
*[[Small round blue cell tumour]] - see ''[[medulloblastoma]]''.
DDx:
*[[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR).<ref name=pmid19563506>{{cite journal |author=Buccoliero AM, Castiglione F, Degl'Innocenti DR, ''et al.'' |title=Embryonal tumor with abundant neuropil and true rosettes: morphological, immunohistochemical, ultrastructural and molecular study of a case showing features of medulloepithelioma and areas of mesenchymal and epithelial differentiation |journal=Neuropathology |volume=30 |issue=1 |pages=84–91 |year=2010 |month=February |pmid=19563506 |doi=10.1111/j.1440-1789.2009.01040.x |url=}}</ref>
Images:
*[http://path.upmc.edu/cases/case414.html Primitive neuroectodermal tumour - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case649.html GBM with PNET component - several images (upmc.edu)].
==Embryonal tumour with abundant neuropil and true rosettes==
*Abbreviated ''ETANTR''.
{{Main|Embryonal tumour with abundant neuropil and true rosettes}}


==CNS lymphoma==
==CNS lymphoma==
Line 499: Line 623:
Image:Primary CNS lymphoma - high mag.jpg | CNS lymphoma - high mag. (WC)
Image:Primary CNS lymphoma - high mag.jpg | CNS lymphoma - high mag. (WC)
Image:Primary CNS lymphoma - very high mag.jpg | CNS lymphoma - very high mag. (WC)
Image:Primary CNS lymphoma - very high mag.jpg | CNS lymphoma - very high mag. (WC)
</gallery>
<gallery>
Image: CNS lymphoma (1) B-cell type.jpg | CNS lymphoma. (WC/KGH)
Image: CNS lymphoma (2) B-cell type.jpg | CNS lymphoma. (WC/KGH)
</gallery>
</gallery>


Line 505: Line 633:


Common pattern:
Common pattern:
*CD20 +ve - key stain.
*[[CD20]] +ve - key stain.
*CD3 -ve.
*CD3 -ve.
*Ki-67 ~40%.
*Ki-67 ~40%.
Line 511: Line 639:
*Bcl-1 -ve.
*Bcl-1 -ve.


==Neurocytoma==
===General===
*Rare.


===Microscopic===
Features:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/Composites/FNA0IE14-Neurocytoma-Micro.htm http://moon.ouhsc.edu/kfung/jty1/Composites/FNA0IE14-Neurocytoma-Micro.htm]. Accessed on: 12 October 2011.</ref>
*[[Pineocytoma|Pineocytomatous]]/neurocytic rosette = irregular rosette with a large meshwork of fibers (neuropil) at the centre.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
**Similar to Homer-Wright rosette.
*Perinuclear clearing.
*Well-defined cell borders.
DDx:
*[[Oligodendroglioma]] - do not have the characteristic rosettes.
*[[Ganglioglioma]].
*[[Ependymoma]].
Images:
*[http://moon.ouhsc.edu/kfung/jty1/Composites/FNA0IE14-Neurocytoma-Micro.htm Neurocytoma (ouhsc.edu)].
*[http://path.upmc.edu/cases/case383.html Neurocytoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case561.html Neurocytoma - cerebellar - several images (upmc.edu)].
===IHC===
*Synaptophysin +ve.
**Most glial tumour -ve.<ref>URL: [http://path.upmc.edu/cases/case383/dx.html http://path.upmc.edu/cases/case383/dx.html]. Accessed on: 15 January 2012.</ref>
==Central neurocytoma==
*Abbreviated ''CNC''.
{{Main|Central neurocytoma}}
==Ganglioglioma==
:'''Not''' to be confused with ''[[ganglioneuroma]]''.
===General===
*Rare.
*Usu. temporal lobe.
*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>
===Microscopic===
Features:
*Atypical neurons.
*Atypical glia.
Images:
*[http://path.upmc.edu/cases/case142.html Ganglioglioma - case 1 (upmc.edu)].
*[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)].
==Lhermitte-Duclos disease==
*Abbreviated ''LDD''.
*[[AKA]] ''dysplastic cerebellar gangliocytoma''.<ref name=pmid20060133>{{Cite journal  | last1 = Yağci-Küpeli | first1 = B. | last2 = Oguz | first2 = KK. | last3 = Bilen | first3 = MA. | last4 = Yalçin | first4 = B. | last5 = Akalan | first5 = N. | last6 = Büyükpamukçu | first6 = M. | title = An unusual cause of posterior fossa mass: Lhermitte-Duclos disease. | journal = J Neurol Sci | volume = 290 | issue = 1-2 | pages = 138-41 | month = Mar | year = 2010 | doi = 10.1016/j.jns.2009.12.010 | PMID = 20060133 }}</ref>
*[[AKA]] ''dysplastic gangliocytoma of the cerebellum''.
{{Main|Lhermitte-Duclos disease}}


==Ganglioneuroblastoma==
==Ganglioneuroblastoma==
Line 574: Line 654:
*Ganglion-like cells with a prominent nucleolus.
*Ganglion-like cells with a prominent nucleolus.
*Small undifferentiated cells with scant cytoplasm.
*Small undifferentiated cells with scant cytoplasm.
 
<gallery>
Image:Adrenal Ganglioneuroblastoma LP CTR.jpg|thumb|Adrenal Ganglioneuroblastoma  - Low power (SKB)
Image:Adrenal Ganglioneuroblastoma MP CTR.jpg|thumb|Adrenal Ganglioneuroblastoma  - Medium power (SKB)
Image:Adrenal Ganglioneuroblastoma HP CTR.jpg|thumb|Adrenal Ganglioneuroblastoma - High power (SKB)
Image:Adrenal Ganglioneuroblastoma HP3 CTR.jpg|thumb|Adrenal Ganglioneuroblastoma - High power (SKB)
Image:Adrenal Ganglioneuroblastoma HP2 CTR.jpg|thumb|Adrenal Ganglioneuroblastoma  - High power (SKB)
</gallery>
Images:
Images:
*[http://path.upmc.edu/cases/case530.html Ganglioneuroblastoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case530.html Ganglioneuroblastoma - several images (upmc.edu)].
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