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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) |
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| #*[[Melanoma]]. | | #*[[Melanoma]]. |
| #*[[Renal cell carcinoma]] (RCC). | | #*[[Renal cell carcinoma]] (RCC). |
| # [[Glioblastoma]] (previously known as ''glioblastoma multiforme''). | | # [[Glioblastoma]], IDH-wildtype. |
| # [[Anaplastic astrocytoma]]. | | # [[Astrocytoma, IDH-mutant]]. |
| # [[Meningioma]]. | | # [[Meningioma]]. |
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| ===Children=== | | ====Children==== |
| # Astrocytoma. | | # [[Pilocytic astrocytoma]]. |
| # [[Medulloblastoma]]. | | # [[Medulloblastoma]]. |
| # [[Ependymoma]]. | | # [[Ependymoma]]. |
| | # Pontine glioma, often [[Diffuse midline glioma, H3 K27-altered]]. |
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| ===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]]. |
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| *[[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]]. |
|
| |
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| ===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. |
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| |
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| ====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. |
|
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| ===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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| |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 |
| |[[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. |
|
| |
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| ==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. |
| <gallery>
| |
| Image:Metastatic_adenocarcinoma_-_cerebellum_-_very_low_mag.jpg | [[CRC]] metastasis to cerebellum - very low mag. (WC)
| |
| Image:Metastatic adenocarcinoma - cerebellum - intermed mag.jpg| CRC metastasis to the cerebellum - intermed. mag. (WC)
| |
| Image:Metastatic_adenocarcinoma_-_cerebellum_-_high_mag.jpg | CRC metastasis to cerebellum - high mag. (WC)
| |
| Image:Brain metastasis - high mag.jpg | Brain metastasis - high mag. (WC)
| |
| </gallery>
| |
|
| |
|
| ===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'''. |
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| *[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> |
| {{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) |
| | * [[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]] |
|
| |
|
| ===General=== | | ===Desmoplastic infantile astrocytoma / Desmoplastic infantile ganglioglioma=== |
| *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> | | * Abbreviated ''DIA'' or ''DIG''. |
| *Paediatric population. | | * 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> |
|
| |
|
| ===Gross/radiology=== | | ===Cerebellar liponeurocytoma=== |
| *Temporal lobe. | | * Previously called ''lipomatous medulloblastoma'' (name changed in WHO 2000). |
| *Variable architecture:<ref name=pmid18071981/> cystic, solitary nodular, multinodular. | | * 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 |
|
| |
|
| ===Microscopic=== | | ====Histo==== |
| 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>
| | * Advanced neuronal and lipomatous differentiation. |
| *Cells similar to oligodendrocytes: | | * Neurocytes: round to oval nuclei with clear cytoplasm. |
| **Large central nuclei with indentations.
| | * Quite cellular. |
| **Multiple small nucleoli (common). | | * Mitoses almost absent. |
| **Clear cytoplasm. | |
|
| |
|
| DDx:
| | ====IHC==== |
| *[[Oligodendroglioma]]. | | * [[GFAP]] +/-ve (focal). |
| **These have rounder, smaller nuclei with occasional nucleoli.<ref name=pmid18071981/> | | * [[MAP2]] +ve. |
| | * Synaptophysin +ve. |
| | * NeuN +ve. |
| | * MIB-1: usu 1-3%. |
|
| |
|
| Images:
| | ====Molecular==== |
| *[http://commons.wikimedia.org/wiki/File:DNET_HE.jpg DNT (WC)]. | | * Distinct methylation profile. |
| *[http://path.upmc.edu/cases/case106.html DNT - several images (upmc.edu)].
| | * 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> |
|
| |
|
| ==Subependymal giant cell astrocytoma==
| | <gallery> |
| *Abbreviated ''SEGA''.
| | File:Cerebellar liponeurocytoma.jpg | Liponeurocytoma, HE (WC/Marvin101). |
| | 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> |
|
| |
|
| ===General=== | | ====DDx==== |
| *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> | | * [[Medulloblastoma]] |
| *WHO Grade I. | | * [[Neurocytoma]] |
|
| |
|
| ===Gross/radiology=== | | ===Gangliocytoma=== |
| *Well-demarcated. | | * Grade I WHO neuronal tumour. |
| | ** ICD-O code: 9492/0 |
| | * Groups of irregular large neurons. |
| | * Non-neoplastic, reticulin-rich glial stroma. |
|
| |
|
| ===Microscopic=== | | ===Ganglioglioma=== |
| 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>
| | :'''Not''' to be confused with ''[[ganglioneuroma]]''. |
| *Giant cells with nuclear atypia ("bizarre cells"). | | ====General==== |
| **Vesicular nuclei.
| | *Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1). |
| **[[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>
| | *Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3) |
| *Glassy eosinophilic cytoplasm.
| | *Rare (approx. 0.5% of all CNS tumors). |
| *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> | | *Usu. temporal lobe. |
| | | *Predominantly children (mean age: 9 years). |
| Images:
| | *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> |
| *[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> | | *Favourable prognosis (survival rates up to 97%) |
| *[http://path.upmc.edu/cases/case500.html SEGA - another case (upmc.edu)].
| | **Insufficient data für anaplastic ganglioglioma. |
| | |
| ===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>
| |
| *Rosenthal fibres are absent - '''key negative'''.
| |
| *Monophasic (unlike classical pilocytic astrocytomas) - '''key negative'''.
| |
| *May rarely have eosinophilic granular bodies. | |
|
| |
|
| ===Grading=== | | ====Macroscopic==== |
| *''WHO Grade II'' by definition.<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref> | | *Circumscribed lesion. |
| | *Usu. contrast enhancing. |
| | *Solid, but intracortical cysts may be present. |
| | *Little mass effect. |
|
| |
|
| ==Atypical teratoid/rhabdoid tumour==
| |
| :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=== | | ====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). |
| Note:
| | File:RGNT HE 2.jpg | RGNT, higher magnification (WC/jensflorian). |
| *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> | | </gallery> |
| | |
| DDx:
| |
| *[[Neurocytoma]] also have perinuclear clearing and well-defined cellular borders.
| |
| **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:
| |
| *[[WC]]: | |
| **[http://commons.wikimedia.org/wiki/File:Oligodendroglioma1_high_mag.jpg Oligodendroglioma high mag. (WC)]. | |
| **[http://commons.wikimedia.org/wiki/File:Oligodendroglioma1_low_mag.jpg Oligodendroglioma low mag. (WC)].
| |
| *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====
| |
| Come in two flavours:
| |
| # WHO grade II.
| |
| #*This is most oligodendrogliomas.
| |
| # WHO grade III.
| |
| #*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=== | | ===Polymorphous low-grade tumor of the young (PLNTY)=== |
| Features:
| | * [[Pediatric-type diffuse low-grade glioma#Diffuse low-grade glioma, MAPK pathway-altered|Polymorphous low-grade tumor of the young (PLNTY)]] |
| *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=== | | ==Pineal tumours== |
| 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>
| | {{Main|Pineal gland}} |
| *Greater chemosensitivity
| |
| *Better prognosis.
| |
|
| |
|
| ==Oligoastrocytoma==
| | * [[Pineocytoma]] |
| ===General===
| | * [[Pineal parenchymal tumour of intermediate differentiation]] |
| *Mixed tumour. | | * [[Pineoblastoma]] |
| | * [[Papillary tumour of the pineal region]] |
|
| |
|
| ===Microscopic=== | | ==Embryonal tumours== |
| Features:
| | * [[Atypical teratoid/rhabdoid tumour]] (AT/RT) or (AT-RT) |
| *Astrocytoma-like and oligodendroglioma-like: | | * [[Medulloblastoma]] |
| *#Oligodendroglioma-like cells = round nucleus, peri-nuclear clearing. | | * [[Primitive neuroectodermal tumour]] (PNET) |
| *#Astrocytoma-like cells = non-ovoid/elongated nucleus. | | * [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR) |
|
| |
|
| 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== | | ===Ganglioneuroma=== |
| | |
| {{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===
| |
| Features:
| |
| *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==
| |
| :'''Not''' to be confused with ''[[ganglioglioma]]''. | | :'''Not''' to be confused with ''[[ganglioglioma]]''. |
| *[[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]].
| |
|
| |
|
| Classification:
| |
| *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>
| |
| **Ganglioneuroma (benign).
| |
| **[[Ganglioneuroblastoma]] (intermediate).
| |
| **[[Neuroblastoma]] (aggressive).
| |
|
| |
|
| ===Gross===
| |
| *Solid.
| |
| *White.
| |
| *Firm.
| |
| *Well-circumscribed.
| |
| *May be nodular.
| |
|
| |
|
| Images:
| | ==Meningioma== |
| *[http://commons.wikimedia.org/wiki/File:Adrenal_ganglioneuroma_02.JPG Adrenal ganglioneuroma (WC)].
| | {{Main|Meningioma}} |
| *[http://www.webpathology.com/image.asp?case=84&n=1 Ganglioneuroma (webpathology.com)].
| |
| | |
| ===Microscopic===
| |
| Features:
| |
| *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]]''.
| |
| | |
| ===IHC===
| |
| Features:<ref>{{Ref GLP|217}}</ref>
| |
| *Spindle cells: S-100 +ve.
| |
| *Ganglion cells: NSE, synaptophysin, NF.
| |
| | |
| ==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====
| |
| www:
| |
| *[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>
| |
| <gallery>
| |
| Image:Subependymoma_-_intermed_mag.jpg | Subependyoma - intermed. mag. (WC)
| |
| Image:Subependymoma_-_high_mag.jpg | Subependymoma - high mag. (WC)
| |
| </gallery>
| |
| | |
| ==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== |
Line 794: |
Line 596: |
| ==Hemangioblastoma== | | ==Hemangioblastoma== |
| {{Main|Hemangioblastoma}} | | {{Main|Hemangioblastoma}} |
|
| |
| ==Medulloblastoma==
| |
| ===General===
| |
| *Mostly paediatric population.
| |
| *May be seen as a component of [[nevoid basal cell carcinoma syndrome]] (NBCCS).
| |
| **Gene: ''patched'' (abbreviated ''PTCH1'').<ref name=omim601309>{{OMIM|601309}}</ref>
| |
| *Commonly spread via cerebrospinal fluid (CSF).<ref>{{Ref APBR|424 Q34}}</ref>
| |
| **May be detected in [[CSF cytopathology]] specimens.
| |
|
| |
| ===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 903: |
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 909: |
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 915: |
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====
| |
| <gallery>
| |
| Image:Central_neurocytoma_-_intermed_mag.jpg | Central neurocytoma - intermed. mag. (WC)
| |
| Image:Central_neurocytoma_-_high_mag.jpg | Central neurocytoma - oligodendrogllioma-like area - high mag. (WC)
| |
| Image:Central_neurocytoma_-_2_-_high_mag.jpg | Central neurocytoma - pseudorosettes - high mag. (WC)
| |
| Image:Central_neurocytoma_-_2_-_very_high_mag.jpg | Central neurocytoma - pseudorossettes - very high mag. (WC)
| |
| </gallery>
| |
| [[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===
| |
| *MIB1 - 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 1,026: |
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)]. |