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Neuropathology tumours (view source)

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[[Image:Gemistocytic Astrocytoma 003.jpg|thumb|right|A brain stem [[astrocytoma]]. (WC)]]
The article covers '''tumours in neuropathology'''.  Tumours are a large part of [[neuropathology]].  [[Cytopathology]] of CNS tumours is dealt with in the article ''[[CNS cytopathology]]''.
The article covers '''tumours in neuropathology'''.  Tumours are a large part of [[neuropathology]].  [[Cytopathology]] of CNS tumours is dealt with in the article ''[[CNS cytopathology]]''.


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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)
Line 11: Line 16:
#*[[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 45: Line 61:
*[[Schwannoma]].
*[[Schwannoma]].
*[[Paraganglioma]].
*[[Paraganglioma]].
 
====Meninges====
====Cerebellopontine angle====
* [[Meningioma]].
DDx:<ref>R. Kiehl. 8 November 2010.</ref>
* [[Solitary fibrous tumour]] / Hemangiopericytoma.
*[[Schwannoma]].
* [[Hemangioblastoma]].
*[[Meningioma]].
less common:
*[[Dermoid cyst]]/epidermoid cyst.
* [[Melanoma]] / Melanocytoma.
*[[Ependymoma]].
* Lymphoproliferative diseases.
*[[Choroid plexus papilloma]].
* [[Sarcoidosis]]
 
* [[Arachnoid cyst]].
===Cystic tumours===
* Disseminated oligodendroglial-like leptomeningeal tumour.
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>
* Desmoplastic infantile astrocytoma / ganglioglioma.
*[[Pilocytic astrocytoma]].  
* Meningioangiomatosis.
*[[Pleomorphic xanthoastrocytoma]].  
* Calcifying pseudoneoplasm.
*[[Ganglioglioma]].
====Skull====
*[[Hemangioblastoma]].
* [[Fibrous dysplasia]].
*[[Craniopharyngioma]].<ref>URL: [http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral]. Accessed on: 14 January 2012.</ref>
* [[Paget disease]].
* [[Histiocytosis]].
* [[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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|missed lesion?
|missed lesion?
|nil
|nil
|[http://commons.wikimedia.org/wiki/File:Grey_matter_and_white_matter_-_very_high_mag.jpg very high mag.], [http://commons.wikimedia.org/wiki/File:Grey_matter_and_white_matter_-_high_mag.jpg high mag.]
|[[Image:Grey_matter_and_white_matter_-_very_high_mag.jpg |thumb|center|150px|Normal. (WC)]]
|-
|-
|[[Reactive astrocytes]]
|[[Reactive astrocytes]]
Line 108: Line 202:
|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
|[http://commons.wikimedia.org/wiki/File:Reactive_astrocytes_-_lfb_-_high_mag.jpg high mag.]
|[[Image:Reactive_astrocytes_-_lfb_-_high_mag.jpg|thumb|center|150px|Reactive astrocytes. (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, 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)]]
|-
|-
|[[Astrocytoma]] (grade II or worse)
|[[Glioblastoma]], IDH-wildtype (CNS [[WHO]] grade 4)
|glial processes (esp. on smear), nuclear atypia (size var. ~3x, irreg. nuc. membrane, hyperchromasia), no Rosenthal fibres in the core of the lesion †
|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+
|[http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma_-_high_mag.jpg high mag.], [http://commons.wikimedia.org/wiki/File:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg very high mag.]
| [[Image:Glioblastoma (1).jpg | thumb| center| 150px|Glioblastoma. (WC)]]
|-
|-
|[[Metastatic brain tumours|Metastasis]]
|[[Metastatic brain tumours|Metastasis]]
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|usu. old
|usu. old
|often suspected to have metastatic disease
|often suspected to have metastatic disease
|TTF-1, CK7, CK20, BRST-2
|[[TTF-1]], CK7, [[CK20]], BRST-2
|[http://commons.wikimedia.org/w/index.php?title=File:Metastatic_adenocarcinoma_-_cerebellum_-_very_low_mag.jpg very low mag.], [http://commons.wikimedia.org/w/index.php?title=File:Metastatic_adenocarcinoma_-_cerebellum_-_high_mag.jpg high mag.]
|[[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: choroid plexus, schwannoma
|EMA, PR, Ki-67
|[http://commons.wikimedia.org/wiki/File:Meningioma_intermed_mag.jpg intermed. mag.]
|-
|[[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
|S100
|[http://commons.wikimedia.org/wiki/File:Schwannoma_-_Antoni_A_and_B_-_intermed_mag.jpg intermed. mag.],
[http://commons.wikimedia.org/wiki/File:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg very high mag.]
|}
|}
† 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.


==Metastatic brain tumours==
==Brain metastasis==
{{Main|Metastases}}
{{Main|Brain metastasis}}
===General===
*Most common brain tumour in adults.
**Usually carcinomas - commonly [[lung cancer|lung]], [[invasive breast cancer|breast]], [[colorectal carcinoma|colon/rectum]].


===Microscopic===
Appearance varies by subtype.


Features of metastatic carcinoma:
===Molecular===
*Tubule formation/glands.
See also: [[Molecular_pathology_tests#Neuropathology|Molecular Neuropathology]]
*Usually well-circumscribed/sharply demarcated from surrounding tissue.
*Usually nuclear atypia including:
**Nuclear hyperchromasia.
**Variation of nuclear size.
**Variation of nuclear shape.
*Mitoses - common.


DDx:
==Gliomas==
*Primary brain tumour - ''see [[Neuropathology_tumours#Primary_versus_secondary|primary brain tumour versus secondary brain tumour]]''.
{{Main|Glioma}}


Images:
Gliomas, glioneuronal tumours and neuronal tumours are often categorized together.
*[http://commons.wikimedia.org/wiki/File:Metastatic_adenocarcinoma_-_cerebellum_-_very_low_mag.jpg CRC metastasis to cerebellum - very low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Metastatic_adenocarcinoma_-_cerebellum_-_high_mag.jpg CRC metastasis to cerebellum - high mag. (WC)].


===IHC===
===Astrocytic tumours===
{{Main|Immunohistochemistry}}
*Carcinoma: pankeratin +ve.
**[[Lung adenocarcinoma]] and SCLC: TTF-1 +ve, CK7 +ve, CK20 -ve.
**Breast carcinoma: CK7 +ve, ER +ve, PR +ve, BRST2 +ve/-ve.
**Colorectal carcinoma: CK7 -ve, CK20 +ve, CDX2 +ve, TTF-1 -ve.
 
==Infiltrative astrocytomas==  
{{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 202: 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 pales forming a defense 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>
===General===
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
*Low-grade astrocytoma.  
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
*Classically in the cerebellum in children; most common glioma in children.<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref>
File:Astroblastoma.jpg | Astroblastoma (AFIP)
*The ''optic glioma'' associated with neurofibromatosis 1.
</gallery>


===Gross===
====Chordoid glioma of the third ventricle====
Features:<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref>
* WHO grade II.
*Usually well-circumscribed.
* Slowly growing, non-invasive, in adults.
*Cystic ''or'' solid.
* Clusters of epithelioid cells in mucinous stroma.
*Do '''not''' smear. (Ref. ?)
* 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>


===Microscopic===
<gallery>
Features:<ref name=Ref_PSNP82-4>{{Ref PSNP|82-4}}</ref>
File:NP op 20201028 009.jpg | Chordoid Glioma. (WC/jensflorian)
*Classically biphasic (though either may be absent):
</gallery>
*#Fibrillar.
*#Microcystic/loose.
*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>
**Best seen on smear or with GFAP [[IHC]].
*Rosenthal fibres - '''key feature'''.
**May be rare.  Not pathognomonic (see below).
*Eosinophilic granular bodies.
*Low cellularity - when compared to medulloblastoma and ependymoma.


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


DDx (of Rosenthal fibers):<ref>MUN. 9 Mar 2009.</ref>
===Ependymal tumours===
*Chronic reactive gliosis.
* [[Subependymoma]]
*Subependymoma.
* [[Myxopapillary Ependymoma]]
*Ganglioma.
* [[Ependymoma]]
*Alexander's disease (rare leukodystrophy).
* Anaplastic ependymoma


DDx of pilocystic astrocytoma (brief):
==Choroid plexus tumours==
*Piloid gliosis.
* [[Choroid plexus papilloma]]
*Oligodendroglioma.
* Atypical choroid plexus papilloma
*Glioblastoma (uncommon - but important).
* [[Choroid plexus carcinoma]]


Images:
==Other neuroepithelial tumours==
*Smears:
* [[Neuropathology_tumours#Cribiform_neuroepithelial_tumour|Cribifiorm neuroepithelial tumour]].
**[http://commons.wikimedia.org/wiki/File:Pilocytic_astrocytoma_-_smear_-_very_high_mag.jpg Bipolar cells with hair-like processes - smear - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Pilocytic_Micro.jpg EGBs - smear (WC/AFIP)].
**[http://commons.wikimedia.org/wiki/File:Rosenthal_fibers.jpg Rosenthal fibres - smear (WC/AFIP)].
*Sections:
**[http://commons.wikimedia.org/wiki/File:Rosenthal_HE_40x.jpg Rosenthal fibres (WC)].
**[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)].


===IHC/special stains===
Features:<ref name=Ref_PSNP84>{{Ref PSNP|84}}</ref>
*GFAP +ve (fibres).
*PAS-D: eosinophilic granular bodies +ve.
*CD68: may have a significant macrophage component.
*KI-67: may be "high" (~20% ???).


===Grading===
===Cribiform neuroepithelial tumour===
*''WHO Grade I'' by definition.
AKA: '''CRINET'''.
*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>


==Pleomorphic xanthoastrocytoma==
==Neuronal and mixed neuronal/glial tumours==
*Abbreviated ''PXA''.
* [[Desmoplastic infantile astrocytoma]] / ganglioglioma (DIA/DIG)
===General===
* [[Dysembryoplastic neuroepithelial tumour]]
Features:
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]]
*Classically in the temporal lobe in children and young adults.
* Cerebellar liponeurocytoma
*Associated with seizures.
* [[Papillary glioneuronal tumour]] (PGNT)
* [[Rosette-forming glioneuronal tumour of the fourth ventricle]] (RGNT)
* Gangliocytoma / Ganglioglioma
* Dysplastic ganglioglioma of the cerebellum ([[Lhermitte-Duclos disease]])
* [[Paraganglioma]]


===Microscopic===
===Desmoplastic infantile astrocytoma / Desmoplastic infantile ganglioglioma===
Features:<ref name=Ref_PBoD8_1333>{{Ref PBoD8|1333}}</ref>
* Abbreviated ''DIA'' or ''DIG''.
*Marked nuclear atypia.
* ICD-O code: 9412/1
*Inflammation (chronic).
* 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>


Notes:
===Cerebellar liponeurocytoma===
*No mitoses.
* Previously called ''lipomatous medulloblastoma'' (name changed in WHO 2000).
*No necrosis.
* 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


Images:
====Histo====
*[http://path.upmc.edu/cases/case499.html Pleomorphic xanthoastrocytoma - several images (upmc.edu)].
* Advanced neuronal and lipomatous differentiation.
*[http://path.upmc.edu/cases/case511.html Pleomorphic xanthoastrocytoma - another case - several images (upmc.edu)].
* Neurocytes: round to oval nuclei with clear cytoplasm.
*[http://path.upmc.edu/cases/case578.html Pleomorphic xanthoastrocytoma - case 3 - several images (upmc.edu)].
* Quite cellular.
*[http://path.upmc.edu/cases/case579.html Pleomorphic xanthoastrocytoma - case 4 - several image (upmc.edu)].
* Mitoses almost absent.  


===IHC===
====IHC====
*GFAP +ve.
* [[GFAP]] +/-ve (focal).
*CD68 +ve.
* [[MAP2]] +ve.
* Synaptophysin +ve.
* NeuN +ve.
* MIB-1: usu 1-3%.


==Dysembryoplastic neuroepithelial tumour==
====Molecular====
*Abbreviated ''DNT''.
* 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>


===General===
<gallery>
*Common tumour cause of drug resistant epilepsy.<ref name=pmid15881751>{{Cite journal  | last1 = Cataltepe | first1 = O. | last2 = Turanli | first2 = G. | last3 = Yalnizoglu | first3 = D. | last4 = Topçu | first4 = M. | last5 = Akalan | first5 = N. | title = Surgical management of temporal lobe tumor-related epilepsy in children. | journal = J Neurosurg | volume = 102 | issue = 3 Suppl | pages = 280-7 | month = Apr | year = 2005 | doi = 10.3171/ped.2005.102.3.0280 | PMID = 15881751 }}</ref>
File:Cerebellar liponeurocytoma.jpg | Liponeurocytoma, HE (WC/Marvin101).
*Paediatric population.
File:Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/Marvin101).
File:Cerebellar Liponeurocytoma HE.jpg | Liponeurocytoma, HE (WC/jensflorian).
File:Cerebellar Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/jensflorian).
</gallery>


===Gross/radiology===
====DDx====
*Temporal lobe.
* [[Medulloblastoma]]
*Variable architecture:<ref name=pmid18071981/> cystic, solitary nodular, multinodular.
* [[Neurocytoma]]


===Microscopic===
===Gangliocytoma===
Features:<ref name=pmid18071981>{{Cite journal  | last1 = O'Brien | first1 = DF. | last2 = Farrell | first2 = M. | last3 = Delanty | first3 = N. | last4 = Traunecker | first4 = H. | last5 = Perrin | first5 = R. | last6 = Smyth | first6 = MD. | last7 = Park | first7 = TS. | title = The Children's Cancer and Leukaemia Group guidelines for the diagnosis and management of dysembryoplastic neuroepithelial tumours. | journal = Br J Neurosurg | volume = 21 | issue = 6 | pages = 539-49 | month = Dec | year = 2007 | doi = 10.1080/02688690701594817 | PMID = 18071981 }}</ref>
* Grade I WHO neuronal tumour.
*Cells similar to oligodendrocytes:
** ICD-O code: 9492/0
**Large central nuclei with indentations.
* Groups of irregular large neurons.
**Multiple small nucleoli (common).
* Non-neoplastic, reticulin-rich glial stroma.
**Clear cytoplasm.


DDx:
===Ganglioglioma===
*[[Oligodendroglioma]].
:'''Not''' to be confused with ''[[ganglioneuroma]]''.
**These have rounder, smaller nuclei with occasional nucleoli.<ref name=pmid18071981/>
====General====
 
*Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1).
Images:
*Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3)
*[http://commons.wikimedia.org/wiki/File:DNET_HE.jpg DNT (WC)].
*Rare (approx. 0.5% of all CNS tumors).
*[http://path.upmc.edu/cases/case106.html DNT - several images (upmc.edu)].
*Usu. temporal lobe.
 
*Predominantly children (mean age: 9 years).  
==Subependymal giant cell astrocytoma==
*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>
*Abbreviated ''SEGA''.
*Favourable prognosis (survival rates up to 97%)
 
**Insufficient data für anaplastic ganglioglioma.
===General===
*Associated with [[tuberous sclerosis complex]] (TSC).<ref name=pmid21455842>{{Cite journal  | last1 = Grajkowska | first1 = W. | last2 = Kotulska | first2 = K. | last3 = Jurkiewicz | first3 = E. | last4 = Roszkowski | first4 = M. | last5 = Daszkiewicz | first5 = P. | last6 = Jóźwiak | first6 = S. | last7 = Matyja | first7 = E. | title = Subependymal giant cell astrocytomas with atypical histological features mimicking malignant gliomas. | journal = Folia Neuropathol | volume = 49 | issue = 1 | pages = 39-46 | month =  | year = 2011 | doi =  | PMID = 21455842 }}</ref>
*WHO Grade I.
 
===Gross/radiology===
*Well-demarcated.
 
===Microscopic===
Features:<ref name=upmc_case179/><ref name=pmid9595853>{{Cite journal  | last1 = Taraszewska | first1 = A. | last2 = Kroh | first2 = H. | last3 = Majchrowski | first3 = A. | title = Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases. | journal = Folia Neuropathol | volume = 35 | issue = 3 | pages = 181-6 | month =  | year = 1997 | doi =  | PMID = 9595853 }}</ref>
*Giant cells with nuclear atypia ("bizarre cells").
**Vesicular nuclei.
**[[Nuclear pseudoinclusions]].<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref>
*Glassy eosinophilic cytoplasm.
*Abundant [[mast cell]]s.<ref name=upmc179>URL: [http://path.upmc.edu/cases/case179/micro.html http://path.upmc.edu/cases/case179/micro.html]. Accessed on: 8 January 2012.</ref>
 
Images:
*[http://path.upmc.edu/cases/case179/micro.html SEGA (upmc.edu)].<ref name=upmc_case179>URL: [http://path.upmc.edu/cases/case179.html http://path.upmc.edu/cases/case179.html]. Accessed on: 29 July 2011.</ref>
*[http://path.upmc.edu/cases/case500.html SEGA - another case (upmc.edu)].
 
===IHC===
Features:<ref name=pmid9595853/>
*GFAP +ve. (???)
*Vimentin +ve. (???)
*S100 +ve. (???)
 
==Pilomyxoid astrocytoma==
===General===
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*A variant of ''pilocytic astrocytoma''.  
**Some have suggested it is a unique entity.<ref name=pmid16048293>{{cite journal |author=Komotar RJ, Mocco J, Jones JE, ''et al.'' |title=Pilomyxoid astrocytoma: diagnosis, prognosis, and management |journal=Neurosurg Focus |volume=18 |issue=6A |pages=E7 |year=2005 |month=June |pmid=16048293 |doi= |url=}}</ref>
*Childhood or adolescence.
 
===Gross===
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*Classically - hypothalamic location/suprasellar location; may involve the sella turcica.<ref name=pmid19766001>{{cite journal |author=Alimohamadi M, Bidabadi MS, Ayan Z, Ketabchi E, Amirjamshidi A |title=Pilomyxoid astrocytoma with involvement of the sella turcica in an adolescent |journal=J Clin Neurosci |volume=16 |issue=12 |pages=1648–9 |year=2009 |month=December |pmid=19766001 |doi=10.1016/j.jocn.2009.01.035 |url=}}</ref>
*Solid.
*Well-circumscribed.
 
===Microscopic===
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*Consists of small round/ovoid bland cells in a [[myxoid stroma]].
*Hair-like fibres ~ 1 micrometer.
**Often difficult to appreciate on standard (H&E) histologic sections.
*Usually angiocentric (surround blood vessel) - '''key feature'''.


Notes:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
====Macroscopic====
*Rosenthal fibres are absent - '''key negative'''.
*Circumscribed lesion.
*Monophasic (unlike classical pilocytic astrocytomas) - '''key negative'''.
*Usu. contrast enhancing.
*May rarely have eosinophilic granular bodies.
*Solid, but intracortical cysts may be present.
*Little mass effect.


===Grading===
*''WHO Grade II'' by definition.<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>


==Atypical teratoid/rhabdoid tumour==
====Microscopic====
:See also: ''[[Extrarenal malignant rhabdoid tumour]]''.
*Commonly abbreviated ''AT/RT''.
*May be written ''atypical teratoid rhabdoid tumour'', i.e. without the forward slash, or ''atypical teratoid-rhabdoid tumour'' (AT-RT).
 
===General===
*Usually supratentorial, occasionally in posterior fossa, case reports of spinal cord.
 
===Microscopic===
Features:
Features:
*Cellular.
*Dysplastic neurons.
*Small round cells usu. with a prominent nucleolus.
**Out of regular architecture / abnormal location.
*Rhabdoid cells.
**Cytomegaly
**Cells with eosinophilic granular cytoplasm + eccentric nucleus.
**Clustering
*Mitoses.
**Binucleated (very occassionally).
*+/-[[Necrosis]] (common).
*Atypical glia.
*Eosinophilic granular bodies.
*Calcification.
*Prominent capillary network.
*Lymphocytic cuffing.
*May contain some reticulin.
*Glial component may resemble:
**Fibrillary astrocytoma.
**Oligodendroglioma.
**Pilocytic astrocytoma.


DDx:
Anaplastic ganglioglioma:
*[[Primitive neuroectodermal tumour]] (PNET).
*Brisk mitotic activity
*[[Medulloblastoma]].
*Necrosis
*[[Diffuse astrocytoma]].
*[[Choroid plexus carcinoma]].
*[[Embryonal carcinoma]].


Images:
====IHC====
*[http://commons.wikimedia.org/wiki/File:Rhabdoidtumourcell.jpg Rhabdoid tumour cell (WC)].
*Neurons:
*[http://commons.wikimedia.org/wiki/File:ATRT-HE-Overview.jpg AT/RT (WC)].
**[[MAP2]] +ve
*[http://commons.wikimedia.org/wiki/File:ATRT-INI1.jpg AT/RT -ve for INI1 (WC)].
**Synaptophysin +ve
*[http://path.upmc.edu/cases/case99/micro.html AR/RT (upmc.edu)].
** Neurofilament +ve
*Glia:
**CD34+/-ve
*BRAF V600E +ve (approx. 25%, mainly ganglion cells).


===IHC===
====Molecular====
*BAF-47 -ve ([[AKA]] ''INI1'', [[AKA]] ''SMARCB1'' - the HGNC symbol<ref name=omim601607>{{OMIM|601607}}</ref>) - virtually diagnostic.
*BRAF V600E-mutated(approx. 25%).
**Endothelial cells +ve control.
*IDH1/2 wt.
*S-100 +ve.
*No 1p/19q codeletion.
**Few other brain tumours express it.
*Usu. Chr. 7 gain.  
*Vimentin +ve (perinuclear condensation).
*CDKN2A deletions in anaplastic ganglioglioma.  


Others:
====DDx:====
*GFAP +ve (focal - in tumour cells).
*[[DNT]].
*EMA +ve (patchy cytoplasmic).
*[[Oligodendroglioma]].
*Smooth muscle actin +ve.
*Trapped cortical neurons in diffuse astrocytoma.
*Papillary glioneuronal tumor.
*Dysembryoplastic neuroepithelial tumor.


==Oligodendroglioma==
====Images====
===General===
<gallery>
*Do ''not'' arise from oligodendrocytes.
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian)
**Arise from ''glial precursor cells''.
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)].


Usual location:
===Lhermitte-Duclos disease===
*Fourth ventricle.
*Abbreviated ''LDD''.
*Intramedullary spinal cord.
*[[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>


Prognosis by flavours (average survival):<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
===Papillary glioneuronal tumour===
*WHO grade II: 10-15 years.
* Abbreviated ''PGNT''.
*WHO grade III: 3-5 years.
* A benign, supratentorial tumor of childhood.
** Biologic course corresponds to WHO grade I.
** 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''.
*Highly cellular lesion composed of:
* Provisional ICD-O code: 9509/1
**Cells resembling ''fried eggs'' (oligodendrocytes) with:
* A rare benign infratentorial tumour of the midline of children and adults.  
***Round nucleus - '''key feature'''.
* Biologic course corresponds to WHO grade I.
***Distinct cell borders.
* Glial component corresponds to [[pilocytic astrocytoma]].
***Moderate-to-marked nuclear atypia.
* Neurocytic rosettes.
***Clear cytoplasm - useful feature (if present).
* Eosinopil fibrillary cores / pseudorosettes.
****Some oligodendrogliomas have eosinophilic cytoplasm with focal perinuclear clearing.
* GFAP+ in fibrillary areas, Syn+ in rosettes.
**Acutely branched capillary sized vessels - "chicken-wire" like appearance.
* Neurocytic cells: MAP2+
***Abundant, delicate appearing; may vaguely resemble a paraganglioma at low power.
* MIB-1 usu. below 3%.
*Calcifications - important feature.<ref>URL: [http://www.emedicine.com/radio/topic481.htm http://www.emedicine.com/radio/topic481.htm].</ref>
<gallery>
File:Histology RGNT HE.jpg | RGNT, HE stain (WC/jensflorian).
File:RGNT HE 2.jpg | RGNT, higher magnification (WC/jensflorian).
</gallery>


Note:
===Polymorphous low-grade tumor of the young (PLNTY)===
*Tumour cells may be plasmacytoid, i.e. have a [[plasma cell]]-like appearance.<ref name=pmid17284109>{{Cite journal  | last1 = Aldape | first1 = K. | last2 = Burger | first2 = PC. | last3 = Perry | first3 = A. | title = Clinicopathologic aspects of 1p/19q loss and the diagnosis of oligodendroglioma. | journal = Arch Pathol Lab Med | volume = 131 | issue = 2 | pages = 242-51 | month = Feb | year = 2007 | doi = 10.1043/1543-2165(2007)131[242:CAOQLA]2.0.CO;2 | PMID = 17284109 | URL = http://www.archivesofpathology.org/doi/full/10.1043/1543-2165(2007)131%5B242:CAOQLA%5D2.0.CO;2 }}</ref>
* [[Pediatric-type diffuse low-grade glioma#Diffuse low-grade glioma, MAPK pathway-altered|Polymorphous low-grade tumor of the young (PLNTY)]]


DDx:
==Pineal tumours==
*[[Neurocytoma]] also have perinuclear clearing and well-defined cellular borders.
{{Main|Pineal gland}}
**Pineocytomatous/neurocytic rosettes = (irregular) rosette with a large meshwork of fibers (neuropil) at the centre.
 
Notes:
*Few neural tumours have round nuclei - DDx:
**[[Oligodendroglioma]].
**[[Lymphoma]].
**Clear cell variant of [[ependymoma]].
**[[Germ cell tumour]] (germinoma/dysgerminoma/seminoma).


Images:
* [[Pineocytoma]]
*[[WC]]:
* [[Pineal parenchymal tumour of intermediate differentiation]]
**[http://commons.wikimedia.org/wiki/File:Oligodendroglioma1_high_mag.jpg Oligodendroglioma high mag. (WC)].
* [[Pineoblastoma]]
**[http://commons.wikimedia.org/wiki/File:Oligodendroglioma1_low_mag.jpg Oligodendroglioma low mag. (WC)].
* [[Papillary tumour of the pineal region]]
*www:
**[http://path.upmc.edu/cases/case713.html Oligodendroglioma - several images (upmc.edu)].
**[http://frontalcortex.com/?page=oll&topic=24&qid=864 Oligodendroglioma with plasmacytoid cells (frontalcortex.com)].


====Histologic grading====
==Embryonal tumours==
Come in two flavours:
* [[Atypical teratoid/rhabdoid tumour]] (AT/RT) or (AT-RT)
# WHO grade II.
* [[Medulloblastoma]]
#*This is most oligodendrogliomas.
* [[Primitive neuroectodermal tumour]] (PNET)
# WHO grade III.
* [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR)
#*Features for calling high grade:<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
#**Endothelial hypertrophy.
#***Plump/large endothelial cells.
#**Necrosis.
#**High mitotic rate (6 mitoses/10 HPF for whatever "HPF" means, see [[HPFitis]]).
 
===IHC===
Features:
*MAP-2 +ve.<reF name=pmid12025943>{{cite journal |author=Suzuki SO, Kitai R, Llena J, Lee SC, Goldman JE, Shafit-Zagardo B |title=MAP-2e, a novel MAP-2 isoform, is expressed in gliomas and delineates tumor architecture and patterns of infiltration |journal=J. Neuropathol. Exp. Neurol. |volume=61 |issue=5 |pages=403–12 |year=2002 |month=May |pmid=12025943 |doi= |url=}}</ref>
*GFAP -ve.
**Some subtypes +ve - should not be used to distinguish.<ref name=Ref_PSNP>{{Ref PSNP|98}}</ref>
*EMA +ve.
*IDH-1 -ve. (???).
*p53 -ve.
**Useful for differentiating ''astrocytoma'' vs. ''oligodendroglioma''.
*Ki-67.
 
===Molecular pathology===
Losses of 1p and 19q both helps with diagnosis and is prognostic:<ref name=pmid18565359>{{cite journal |author=Fontaine D, Vandenbos F, Lebrun C, Paquis V, Frenay M |title=[Diagnostic and prognostic values of 1p and 19q deletions in adult gliomas: critical review of the literature and implications in daily clinical practice] |language=French |journal=Rev. Neurol. (Paris) |volume=164 |issue=6-7 |pages=595–604 |year=2008 |pmid=18565359 |doi=10.1016/j.neurol.2008.04.002 |url=}}</ref>
*Greater chemosensitivity
*Better prognosis.
 
==Oligoastrocytoma==
===General===
*Mixed tumour.
 
===Microscopic===
Features:
*Astrocytoma-like and oligodendroglioma-like:
*#Oligodendroglioma-like cells = round nucleus, peri-nuclear clearing.
*#Astrocytoma-like cells = non-ovoid/elongated nucleus.


DDx:
DDx:
*Anaplastic astrocytoma.
* [[Ewing sarcoma]]
*Oligodendroglioma. (???)
* [[Sarcoma with CIC-rearrangement]]
 
===IHC===
*Oligodendroglioma-like cells: MAP-2 +ve (cytoplasm).
*Astrocytoma-like cells: GFAP +ve (cytoplasm, nuclear membrane).
 
Others:
*Ki-67 ~10%. (???)
*p53 - focally +ve. (???)
*IDH-1 -ve. (???)
 
==Meningioma==
{{Main|Meningioma}}
===General===
*Very common.
*May be part of a syndrome.
 
===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]]''.


==Peripheral nerve sheath tumours==
==Peripheral nerve sheath tumours==
{{Main|Peripheral nerve sheath tumours}}
{{Main|Peripheral nerve sheath tumours}}
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>
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>
*Benign:
'''Benign:'''
**[[Schwannoma]].
*[[Schwannoma]].
**[[Neurofibroma]].
*[[Neurofibroma]].
**[[Perineurioma]].
*[[Perineurioma]].
*Ganglioneuroma.
**[[Traumatic neuroma]].
**[[Traumatic neuroma]].
*Malignant:
'''Malignant:'''
**[[Malignant peripheral nerve sheath tumour]] (MPNST).
*[[Malignant peripheral nerve sheath tumour]] (MPNST).
 
==Schwannoma==
 
{{Main|Schwannoma}}
===General===
*Tumour of tissue surrounding a nerve.
**Axons adjacent to the tumour are normal... but may be compressed.
 
===Microscopic===
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>
*Antoni A:
**Cellular.
**'Fibrillary, polar, elongated'.
*Antoni B:
**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:
*[http://www.pathguy.com/~lulo/lulo0003.htm Antoni A (pathguy.com)].
*[http://www.ajnr.org/cgi/content/full/28/9/1633/F8 Antoni A & Antoni B side-by-side (ajnr.org)].
 
Notes:
*Several subtypes exist.
 
==Neurofibroma==
{{Main|Peripheral nerve sheath tumours#Neurofibroma}}
===General===
*May be a part of [[neurofibromatosis]] 1.
*Composed of Schwann cells, axons, fibrous material.<ref name=pmid17893219/>


===Microscopic===
===Ganglioneuroma===
Features:
:'''Not''' to be confused with ''[[ganglioglioma]]''.
*Spindle cells lesion.
**See ''[[Neurofibroma]]'' article for details.
 
Image:
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(1).jpg Neurofibroma - low mag. (WC)].
*[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==
*[[AKA]] ganglioma.<ref>URL: [http://medical-dictionary.thefreedictionary.com/ganglioma http://medical-dictionary.thefreedictionary.com/ganglioma]. Accessed on: 8 November 2010.</ref>
*[[AKA]] ganglioma.<ref>URL: [http://medical-dictionary.thefreedictionary.com/ganglioma http://medical-dictionary.thefreedictionary.com/ganglioma]. Accessed on: 8 November 2010.</ref>
===General===
{{Main|Ganglioneuroma}}
*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]].


===Gross===
*Solid.
*White.
*Firm.
*Well-circumscribed.
*May be nodular.


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


===Microscopic===
==Meningioma==
Features:
{{Main|Meningioma}}
*Ganglion cells - '''key feature'''.
**Large cells with large nucleus.
***Prominent nucleolus.
*Disordered fibrinous-like material.
*Eosinophilic granular bodies.<ref>R. Kiehl. 8 November 2010.</ref>
 
Images:
*[http://it.wikipedia.org/wiki/File:Ganglioneuroma_(1).jpg Ganglioneuroma (WC)].
*[http://www.webpathology.com/image.asp?n=4&Case=84 Ganglioneuroma (webpathology.com)].
*[http://www.webpathology.com/image.asp?case=412&n=4 Ganglioneuroma (webpathology.com)].
*[http://en.wikipedia.org/wiki/File:Ganglion_high_mag.jpg Normal ganglion - high mag. (WC)] .
 
See: ''[[adrenal ganglioneuroma]]'', ''[[Gastrointestinal tract polyps#Ganglioneuroma|colonic ganglioneuroma]]''.
 
==Ependymoma==
===General===
*Called the forgotten glial tumour.
 
Epidemiology:<ref name=Ref_PBoD8_1334>{{Ref PBoD8|1334}}</ref>
*Usual site:
**Adults: usu. spinal cord.
**Children: usu. posterior fossa.
*May be assoc. with [[neurofibromatosis]] 2.
 
Comes in two main flavours:
#Ependymoma (not otherwise specified).
#Myxopapillary ependymoma.
#*Classically at filum terminale.
 
Other flavours:<ref>URL: [http://emedicine.medscape.com/article/1744030-overview http://emedicine.medscape.com/article/1744030-overview]. Accessed on: 17 January 2012.</ref>
*Papillary ependymoma.
*Clear cell ependymoma.
 
===Microscopic===
====Classic ependymoma====
Features:
*Cells have a "tadpole-like" morphology.
**May also be described as ''ice cream cone-shaped''.<ref>[http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html]</ref>
*'''Rosettes''' = circular nuclear free zones/cells arranged in a pseudoglandular fashion; comes in two flavours in ependymoma:
**''Perivascular pseudorosettes'' = (tumour) cells arranged around a blood vessel; nuclei of cells distant from the blood vessel, i.e. rim of cytoplasm (from tumour cells) surround blood vessel (nucleus-free zone); more common than ependymal rosette... but less specific.
**''Ependymal rosette'' ([[AKA]] ''true ependymal rosette'') = rosette has an empty space at the centre - '''key feature'''.
*Nuclear features monotonous, i.e. "boring".<ref>MUN. 6 Oct 2009.</ref>
**There is little variation in size, shape and staining.
 
DDx (classic ependymoma):
*[[Subependymoma]].
*[[Glioblastoma]] (GBM).
**Invasive border = GBM; circumscribed border of lesion = ependymoma.
 
Images:
*www:
**[http://www.flickr.com/photos/ckrishnan/3862487821/in/photostream Ependymoma (flickr.com)].
**[http://www.ajnr.org/cgi/content-nw/full/27/3/488/F10 Ependymoma - ependymal rosettes (ajnr.org)].
**[http://path.upmc.edu/cases/case95/micro.html Anaplastic ependymoma - case 1 (upmc.edu)].
**[http://path.upmc.edu/cases/case324.html Anaplastic ependymoma - case 2 (upmc.edu)].
*WC:
**[http://commons.wikimedia.org/wiki/File:Ependymoma_intermed_mag.jpg Ependymoma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Ependymoma_low_intermed_mag.jpg Ependymoma - low mag. (WC)].
 
====Myxopapillary ependymoma====
Features:
*Perivascular pseudorosettes:
**Myxoid material surround blood vessels.
***[[Myxoid]] material surrounded by tumour cells.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Myxopapillary_ependymoma_-_high_mag.jpg Myxopapillary ependymoma - high mag. (WC)].
*[http://careers.bmj.com/article-images/cf0708.f2_default.gif Myxopapillary ependymoma (bmj.com)] - part of [http://careers.bmj.com/careers/advice/view-article.html?id=351 careers.bmj.com article on paediatric pathology].
*[http://commons.wikimedia.org/wiki/File:Myxopapillary_ependymoma.jpg Myxopapillary ependymoma - cytology (WC)].
*[http://path.upmc.edu/cases/case626.html Myxopapillary ependymoma - several images (upmc.edu)].
 
====Grading====
Easy:
*Subependymoma = WHO grade I.
*Myxopapillary ependymoma = WHO grade I.
Not-so-easy:
*Classic ependymoma = WHO grade II.
*Anaplastic ependymoma = WHO grade III.
 
Grade II vs. Grade III:
*Cellular density.
*Mitoses.
*Necrosis.
*Microvascular proliferation.
 
Notes:
*Many tumours fall between grade II and grade III.  These are called "indeterminate" by many.
 
===IHC===
*Reticulin.
*GFAP.
*MIB1.
 
==Subependymoma==
===General===
*Good prognosis - WHO Grade I.
 
===Gross/radiology===
*Classic location: fourth ventricle.<ref>{{Cite journal  | last1 = Hoeffel | first1 = C. | last2 = Boukobza | first2 = M. | last3 = Polivka | first3 = M. | last4 = Lot | first4 = G. | last5 = Guichard | first5 = JP. | last6 = Lafitte | first6 = F. | last7 = Reizine | first7 = D. | last8 = Merland | first8 = JJ. | title = MR manifestations of subependymomas. | journal = AJNR Am J Neuroradiol | volume = 16 | issue = 10 | pages = 2121-9 | month =  | year =  | doi =  | PMID = 8585504 |url=http://www.ajnr.org/cgi/reprint/16/10/2121}}</ref>
*Well demarcated margin.
*Usu. completely within the ventricle; does not extend into brain (like [[ependymoma]]s).
 
===Microscopic===
Features:<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
*Microcysts with bluish material - give a spongy appearance at low magnification.
*Nuclei cluster.
**Described as "bundles of flowers".
 
Negatives.
*No nuclear pleomorphism, no prominent nucleoli, no mitoses.
 
Images:
*[http://moon.ouhsc.edu/kfung/jty1/Com05/Com05Image/Com501-2-04.gif Subependymoma (ouhsc.edu)].<ref name=ouhsc>URL: [http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com05/Com501-2-Diss.htm]. Accessed on: 2 June 2011.</ref>
*[http://commons.wikimedia.org/wiki/File:Subependymoma_-_intermed_mag.jpg Subependyoma - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Subependymoma_-_high_mag.jpg Subependymoma - high mag. (WC)].
 
==Choroid plexus papilloma==
===General===
*Benign - WHO grade I.<ref name=pmid20644273>{{Cite journal  | last1 = Menon | first1 = G. | last2 = Nair | first2 = SN. | last3 = Baldawa | first3 = SS. | last4 = Rao | first4 = RB. | last5 = Krishnakumar | first5 = KP. | last6 = Gopalakrishnan | first6 = CV. | title = Choroid plexus tumors: an institutional series of 25 patients. | journal = Neurol India | volume = 58 | issue = 3 | pages = 429-35 | month =  | year =  | doi = 10.4103/0028-3886.66455 | PMID = 20644273 |URL = http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2010;volume=58;issue=3;spage=429;epage=435;aulast=Menon }}</ref>
*Usu. laternal ventricle in kids.<ref>URL: [http://emedicine.medscape.com/article/250795-overview http://emedicine.medscape.com/article/250795-overview]. Accessed on: 3 June 2011.</ref>
 
===Microscopic===
Features:
*Simple epithelium.
*Papillae.
*Psammoma bodies.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Choroid_plexus_papilloma_micrograph.jpg Choroid plexus papilloma (WC)].
*[http://path.upmc.edu/cases/case551.html Choroid plexus papilloma - oncocytic variant - several images (upmc.edu)].
 
==Choroid plexus carcinoma==
===General===
*Usually pediatric population.
*Malignant counterpart of ''[[choroid plexus papilloma]]''.<ref name=pmid19679976>{{Cite journal  | last1 = Singh | first1 = A. | last2 = Vermani | first2 = S. | last3 = Shruti | first3 = S. | title = Choroid plexus carcinoma: report of two cases. | journal = Indian J Pathol Microbiol | volume = 52 | issue = 3 | pages = 405-7 | month =  | year =  | doi = 10.4103/0377-4929.55009 | PMID = 19679976 }}</ref>
*Poor prognosis - WHO grade III.<ref name=pmid20644273>{{Cite journal  | last1 = Menon | first1 = G. | last2 = Nair | first2 = SN. | last3 = Baldawa | first3 = SS. | last4 = Rao | first4 = RB. | last5 = Krishnakumar | first5 = KP. | last6 = Gopalakrishnan | first6 = CV. | title = Choroid plexus tumors: an institutional series of 25 patients. | journal = Neurol India | volume = 58 | issue = 3 | pages = 429-35 | month =  | year =  | doi = 10.4103/0028-3886.66455 | PMID = 20644273 |URL = http://www.neurologyindia.com/article.asp?issn=0028-3886;year=2010;volume=58;issue=3;spage=429;epage=435;aulast=Menon }}</ref>
*Classically posterior fossa.
*Intraventricular mass.
 
===Microscopic===
Features:<ref name=pmid19679976/>
*Choroid plexus epithelium with [[nuclear pleomorphism]] & high [[NC ratio]].
*Mitoses.
*Necrosis.
*+/-Brain invasion.
 
DDx:
*[[Choroid plexus papilloma]].
*Atypical plexus papilloma - has features intermediate between ''choroid plexus papilloma'' and ''choroid plexus carcinoma''.<ref name=pmid20644273/>
*[[Atypical teratoid/rhabdoid tumour]].
 
Images:
*[http://path.upmc.edu/cases/case198/micro.html Choroid plexus carcinoma - several images (upmc.edu)].
 
===IHC===
Features:<ref name=pmid20644273/>
*Cytokeratins +ve.
*EMA usu. -ve.
*GFAP -ve (~20% +ve).
*Ki-67 high.
**Useful to diff. from benign counterpart.
*INI1 +ve.


==Chordoma==
==Chordoma==
===General===
{{Main|Chordoma}}
*Location: usually sacrum or clivus.
*It is a [[bone tumour]].
 
===Gross===
*Soft, gelatinous, lobulated.<ref>URL: [http://www.histopathology-india.net/Chordoma.htm http://www.histopathology-india.net/Chordoma.htm]. Accessed on: 12 April 2012.</ref>
 
DDx:
*Bony [[metastasis]] (mucinous carcinoma) - typically multifocal.
 
Image:
*[http://www.flickr.com/photos/bc_the_path/4428268879/in/photostream/ Chordoma (flickr.com)].
 
===Microscopic===
Features:<ref name=Ref_DCHH184>{{Ref DCHH|184}}</ref>
*Architecture: islands of cells surrounded by fibrous tissue.
**Also described as "lobulated" architecture; may not be apparent.
*[[Myxoid]] background - grey extracellular material, variable amount present.
*Mixed cell population:
*#Abundant eosinophilic cytoplasm.
*#''Physaliphorous cells'' or ''bubble cells'' - '''key feature'''.
*#*Have a very large clear bubble with a sharp border; bubble does not compress nucleus - nucleus may be in bubble.
 
DDx:
*[[Chondrosarcoma]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Chordoma_-_low_mag.jpg Chordoma - low mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Chordoma_-_high_mag.jpg Chordoma - high mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case64.html Chordoma (upmc.edu)].
**[http://path.upmc.edu/cases/case312/micro.html Chordoma - sacrum - several images (upmc.edu)].
**[http://path.upmc.edu/cases/case689.html Chordoma - additional case with several images (upmc.edu)].
 
===IHC===
Features:<ref>URL: [http://path.upmc.edu/cases/case312/micro.html http://path.upmc.edu/cases/case312/micro.html]. Accessed on: 14 January 2012.</ref><ref name=pmid2431128>{{Cite journal  | last1 = Coindre | first1 = JM. | last2 = Rivel | first2 = J. | last3 = Trojani | first3 = M. | last4 = De Mascarel | first4 = I. | last5 = De Mascarel | first5 = A. | title = Immunohistological study in chordomas. | journal = J Pathol | volume = 150 | issue = 1 | pages = 61-3 | month = Sep | year = 1986 | doi = 10.1002/path.1711500110 | PMID = 2431128 }}</ref>
*S100 +ve.
*AE1/AE3 +ve.
*Brachyury +ve -- '''key stain'''.
**Protein important for axial development, affects notochord development.<ref name=omim601397>{{OMIM|601397}}</ref>
**''Brachyury'' literally means ''short tail''.<ref>URL: [http://www.jstor.org/pss/86845 http://www.jstor.org/pss/86845]. Accessed on: 18 May 2010.</ref>
*EMA +ve.


==Hemangioblastoma==
==Hemangioblastoma==
===General===
{{Main|Hemangioblastoma}}
*Usually ''cerebellar''.
*Associated with [[von Hippel-Lindau syndrome]].
*WHO grade I.<ref>URL: [http://www.expertconsultbook.com/expertconsult/ob/book.do?method=display&type=bookPage&decorator=none&eid=4-u1.0-B978-1-4160-4580-9..00019-8--sc0155&isbn=978-1-4160-4580-9 http://www.expertconsultbook.com/expertconsult/ob/book.do?method=display&type=bookPage&decorator=none&eid=4-u1.0-B978-1-4160-4580-9..00019-8--sc0155&isbn=978-1-4160-4580-9]. Accessed on: 9 December 2010.</ref>
 
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/340994-media http://emedicine.medscape.com/article/340994-media]. Accessed on: 23 June 2010.</ref>
*Vascular.
*Polygonal stromal cells with:
**Hyperchromatic nuclei.
**Vacuolar cytoplasm.
 
DDx:
*Metastatic [[clear cell renal cell carcinoma]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Cerebellar_hemangioblastoma_intermed_mag.jpg Hemangioblastoma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Cerebellar_hemangioblastoma_high_mag.jpg Hemangioblastoma - high mag. (WC)].
**[http://commons.wikimedia.org/w/index.php?title=File:Hemangioblastoma_-_nse_-_intermed_mag.jpg Hemangioblastoma - NSE - intermed. mag. (WC)].
*www:
**[http://path.upmc.edu/cases/case342.html Hemangioblastoma - several images (upmc.edu)].
 
===IHC===
Features:<ref>URL: [http://www.nature.com/modpathol/journal/v18/n6/full/3800351a.html http://www.nature.com/modpathol/journal/v18/n6/full/3800351a.html]. Accessed on: 9 December 2010.</ref>
*Alpha-inhibin +ve (cytoplasm).
*EMA -ve.
**RCC typically +ve.
*NSE +ve (nucleus + cytoplasm).
**RCC typically -ve.
 
==Medulloblastoma==
===General===
*Mostly paediatric population.
*May be seen as a component of [[nevoid basal cell carcinoma syndrome]] (NBCCS).
 
===Gross===
*Location: cerebellum - '''key feature'''.
**Morphologically identical supratentorial tumours are called ''[[primitive neuroectodermal tumour]]'' (PNET).
 
===Microscopic===
Features:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm]. Accessed on: 26 October 2010.</ref>
*[[Small round cell tumour]].
*Homer-Wright [[rosette]]s:
**Rosette with a meshwork of fibers (neuropil) at the centre.<ref>{{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>
 
Images:
*[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm Medulloblastoma (ouhsc.edu)].
*[http://path.upmc.edu/cases/case667.html Medulloblastoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case685.html Medulloblastoma with rhabdomyoblastic differentiation - several images (upmc.edu)].
 
DDx:
*[[Small round cell tumours]].
 
====Subtypes====
*Classic medulloblastoma (~85% of all medulloblastomas).
*Variants of medulloblastoma (~15% of all medulloblastomas together):
*#Anaplastic variant.
*#Large cell variant.
*#Desmoplastic/nodular medulloblastoma (DNMB).
*#Medulloblastoma with extensive nodularity (MBEN).
 
Notes:
*Prognosis:<ref name=pmid18841049>{{cite journal |author=Gulino A, Arcella A, Giangaspero F |title=Pathological and molecular heterogeneity of medulloblastoma |journal=Curr Opin Oncol |volume=20 |issue=6 |pages=668–75 |year=2008 |month=November |pmid=18841049 |doi=10.1097/CCO.0b013e32831369f4 |url=}}</ref><ref name=pmid20940197>{{cite journal |author=Rutkowski S, von Hoff K, Emser A, ''et al.'' |title=Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis |journal=J Clin Oncol |volume=28 |issue=33 |pages=4961–4968 |year=2010 |month=November |pmid=20940197 |doi=10.1200/JCO.2010.30.2299 |url=}}</ref> DNMB & MBEN > classic > anaplastic variant, large cell variant.
 
=====Anaplastic variant=====
Features:
*Larger cells.
*Severe anaplasia.
*Polygonal cells.
 
==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''.
===General===
*Super rare.
*Reported only in children <4 years old.<ref name=pmid16551982/>
 
===Microscopic===
Features:<ref name=pmid20499240>{{Cite journal  | last1 = Ferri Niguez | first1 = B. | last2 = Martínez-Lage | first2 = JF. | last3 = Almagro | first3 = MJ. | last4 = Fuster | first4 = JL. | last5 = Serrano | first5 = C. | last6 = Torroba | first6 = MA. | last7 = Sola | first7 = J. | title = Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update. | journal = Childs Nerv Syst | volume = 26 | issue = 8 | pages = 1003-8 | month = Aug | year = 2010 | doi = 10.1007/s00381-010-1179-x | PMID = 20499240 }}</ref>
*[[Small round blue cell tumour]].
*True rosettes = flower-like cluster of cells that surrounds a space.<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>
*Fibrillary neuropil.
**Meshwork of fibers.
 
DDx:
*[[Primitive neuroectodermal tumour]].
*[[Medulloblastoma]]. (???)
 
Images:
*[http://path.upmc.edu/cases/case585.html ETANTR - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case666.html ETANTR - another case (upmc.edu)].


==CNS lymphoma==
==CNS lymphoma==
Line 993: Line 614:
*Perivascular clustering.
*Perivascular clustering.


Images:
====Images====
*www:
www:
**[http://frontalcortex.com/?page=image&topic=1&qid=1237 CNS lymphoma (frontalcortex.com)].
*[http://frontalcortex.com/?page=image&topic=1&qid=1237 CNS lymphoma (frontalcortex.com)].
**[http://path.upmc.edu/cases/case403.html Primary CNS lymphoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case403.html Primary CNS lymphoma - several images (upmc.edu)].
*[[WC]]:
<gallery>
**[http://commons.wikimedia.org/wiki/File:Primary_CNS_lymphoma_-_high_mag.jpg CNS lymphoma - high mag. (WC)].
Image:Primary CNS lymphoma - low mag.jpg | CNS lymphoma - low mag. (WC)
**[http://commons.wikimedia.org/wiki/File:Primary_CNS_lymphoma_-_low_mag.jpg CNS lymphoma - low mag. (WC)].
Image:Primary CNS lymphoma - intermed mag.jpg | CNS lymphoma - intermed. 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)
</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>


===IHC===
===IHC===
Line 1,005: 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 1,011: 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''.
===General===
*Rare - less than 1% of brain tumours.<ref name=pmid16163043>{{Cite journal  | last1 = Chuang | first1 = MT. | last2 = Lin | first2 = WC. | last3 = Tsai | first3 = HY. | last4 = Liu | first4 = GC. | last5 = Hu | first5 = SW. | last6 = Chiang | first6 = IC. | title = 3-T proton magnetic resonance spectroscopy of central neurocytoma: 3 case reports and review of the literature. | journal = J Comput Assist Tomogr | volume = 29 | issue = 5 | pages = 683-8 | month =  | year =  | doi =  | PMID = 16163043 }}</ref>
*Benign.
*First described in 1982.<ref name=pmid16163043/>
===Gross/radiology===
*Intraventricular.<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/Com/Com307-1-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com/Com307-1-Diss.htm]. Accessed on: 12 January 2012.</ref>
**Characteristically attached to the ''septum pellucidum''.<ref name=pmid20692674>{{Cite journal  | last1 = Kerkeni | first1 = A. | last2 = Ben Lakhdher | first2 = Z. | last3 = Rkhami | first3 = M. | last4 = Sebai | first4 = R. | last5 = Belguith | first5 = L. | last6 = Khaldi | first6 = M. | last7 = Ben Hamouda | first7 = M. | title = [Central neurocytoma: Study of 32 cases and review of the literature]. | journal = Neurochirurgie | volume = 56 | issue = 5 | pages = 408-14 | month = Oct | year = 2010 | doi = 10.1016/j.neuchi.2010.07.001 | PMID = 20692674 }}</ref>
===Microscopic===
Features:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/Com/Com307-1-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com/Com307-1-Diss.htm]. Accessed on: 27 May 2011.</ref>
*Perivascular pseudorosette = circular/flower-like arrangement of cells with blood vessel 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>
*Islands of neuropil.
*Polygonal cells with a perinuclear halo.
DDx:
*[[Oligodendroglioma]].


DDx of perivascular pseudorosette:
*Ependymoma.
*[[Medulloblastoma]], PNET.
*[[Glioblastoma]]s.
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Central_neurocytoma_-_intermed_mag.jpg Central neurocytoma - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Central_neurocytoma_-_high_mag.jpg Central neurocytoma - oligodendrogllioma-like area - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Central_neurocytoma_-_2_-_high_mag.jpg Central neurocytoma - pseudorosettes - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Central_neurocytoma_-_2_-_very_high_mag.jpg Central neurocytoma - pseudorossettes - very high mag. (WC)].
*[[www]]:
**[http://moon.ouhsc.edu/kfung/jty1/Com/Com307-1-Diss.htm Central neurocytoma - several images (ouhsc.edu)].
**[http://frontalcortex.com/?page=image&topic=1&qid=1203 Central neurocytoma (frontalcortex.com)].
**[http://path.upmc.edu/cases/case74/micro.html Central neurocytoma - crappy images (upmc.edu)].
===IHC===
*MIB-1 - high may predict re-occurance.<ref name=pmid15015671>{{Cite journal  | last1 = Schmidt | first1 = MH. | last2 = Gottfried | first2 = ON. | last3 = von Koch | first3 = CS. | last4 = Chang | first4 = SM. | last5 = McDermott | first5 = MW. | title = Central neurocytoma: a review. | journal = J Neurooncol | volume = 66 | issue = 3 | pages = 377-84 | month = Feb | year = 2004 | doi =  | PMID = 15015671 }}</ref>
==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''.
===General===
*Cerebellar lesion.
*May be associated with [[Cowden syndrome]].<ref name=omim158350>{{OMIM|158350}}</ref>
===Microscopic===
Features:<ref>URL: [http://path.upmc.edu/cases/case472.html http://path.upmc.edu/cases/case472.html]. Accessed on: 21 January 2012.</ref>
*The outer (molecular) layer has increased cellularity.
*Purkinje cells absent.<ref name=pmid20060133/>
*Large (polygonal) cells with round nuclei and prominent nucleoli in the inner (granular) layer - '''key feature'''.
*+/-Microcalcifications.
Images:
*[http://path.upmc.edu/cases/case472.html LDD - several images (upmc.edu)].


==Ganglioneuroblastoma==
==Ganglioneuroblastoma==
Line 1,121: 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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