Difference between revisions of "Neuropathology tumours"

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(sort + update ganglioglioma (needs split out in future))
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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 16: Line 20:
# [[Meningioma]].
# [[Meningioma]].


===Children===
====Children====
# Pilocytic astrocytoma.
# [[Pilocytic astrocytoma]].
# [[Medulloblastoma]].
# [[Medulloblastoma]].
# [[Ependymoma]].
# [[Ependymoma]].
Line 23: Line 27:
===Location (most common)===
===Location (most common)===
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.
Line 29: Line 33:
**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:
====Spinal cord====
**[[Ependymoma]], glioblastoma.
**[[Ependymoma]], glioblastoma.
**Filum terminale - [[myxopapillary ependymoma]], [[paraganglioma]].
**Filum terminale - [[myxopapillary ependymoma]], [[paraganglioma]].
====Filum terminale====
====Filum terminale====
*Filum terminale = bottom end of the spinal cord - has a limited differential.
*Filum terminale = bottom end of the spinal cord - has a limited differential.
DDx:<ref>JLK. 31 May 2010.</ref>
DDx:<ref>JLK. 31 May 2010.</ref>
*[[Meningioma]].
*[[Meningioma]].
Line 49: Line 51:
====Cerebellopontine angle====
====Cerebellopontine angle====
*Abbreviated ''CP angle''.
*Abbreviated ''CP angle''.
DDx:<ref>R. Kiehl. 8 November 2010.</ref>
DDx:<ref>R. Kiehl. 8 November 2010.</ref>
*[[Schwannoma]].
*[[Schwannoma]].
Line 248: Line 249:
*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>
*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==
==Astrocytic tumours==
{{Main|Pilocytic astrocytoma}}
{{Main|Astrocytoma}}


==Pilomyxoid astrocytoma==
* Diffuse [[Astrocytoma]]
{{Main|Pilomyxoid astrocytoma}}
* [[Anaplastic astrocytoma]]
* [[Glioblastoma]]
* [[Gliomatosis cerebri]]
* [[Pilocytic astrocytoma]] (PA)
* [[Pilomyxoid astrocytoma]] (PMA)
* [[Pleomorphic xanthoastrocytoma]] (PXA)
* [[Subependymal giant cell astrocytoma]] (SEGA)


==Pleomorphic xanthoastrocytoma==
==Oligodendroglial tumours==
*Abbreviated ''PXA''.
* [[Oligodendroglioma]]
{{Main|Pleomorphic xanthoastrocytoma}}
* Anaplastic oligodendroglioma
* [[Oligoastrocytoma]]
* Anaplastic oligoastrocytoma


==Subependymal giant cell astrocytoma==
==Ependymal tumours==
*Abbreviated ''SEGA''.
* [[Subependymoma]]
{{Main|Subependymal giant cell astrocytoma}}
* [[Myxopapillary Ependymoma]]
* [[Ependymoma]]
* Anaplastic ependymoma


==Oligoastrocytoma==
==Choroid plexus tumours==
{{Main|Oligoastrocytoma}}
* [[Choroid plexus papilloma]]
* Atypical choroid plexus papilloma
* [[Choroid plexus carcinoma]]


==Oligodendroglioma==
==Other neuroepithelial tumours==
{{Main|Oligodendroglioma}}
* Astroblastoma
* Chordoid glioma of the third ventricle
* [[Angiocentric glioma]]


==Subependymoma==
===Astroblastoma===
{{Main|Subependymoma}}
*No WHO grade yet.
*Very rare superficial tumor of young age.
*Large, cystic. Pushing margin towards CNS.
*Vasocentric growth, plump cells with absence of fibrillary pattern.
*GFAP+ve, Synaptohysin-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.


==Myxopapillary Ependymoma==
<gallery>
{{Main|Myxopapillary Ependymoma}}
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
 
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
==Ependymoma==
File:Astroblastoma.jpg | Astroblastoma (AFIP)
{{Main|Ependymoma}}
</gallery>
 
==Choroid plexus papilloma==
*Grade I WHO or Grade II WHO (atypical CPP)
{{Main|Choroid plexus papilloma}}
 
==Choroid plexus carcinoma==
{{Main|Choroid plexus carcinoma}}
 
==Angiocentric glioma==
*Grade I WHO neuroepithelial tumour.
{{Main|Angiocentric glioma}}


==Chordoid glioma of the 3rd ventricle==
===Chordoid glioma of the third ventricle===
* WHO grade II.
* WHO grade II.
* Slowly growing, non-invasive.
* Slowly growing, non-invasive.
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* GFAP+ve,  MIB-1 1-3%.
* GFAP+ve,  MIB-1 1-3%.


==Gangliocytoma==
==Neuronal and mixed neuronal/glial tumours==
* Desmoplastic infantile astrocytoma / ganglioglioma (DIA/DIG)
* [[Dysembryoplastic neuroepithelial tumour]]
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]]
* Cerebellar liponeurocytoma
* Papillary glioneuronal tumour
* Rosette-forming glioneuronal tumour of the fourth ventricle
* Gangliocytoma / Ganglioglioma
* Dysplastic ganglioglioma of the cerebellum (Lhermitte-Duclos)
* [[Paraganglioma]]
 
===Gangliocytoma===
* Grade I WHO neuronal tumour.
* Grade I WHO neuronal tumour.
** ICD-O code: 9492/0
** ICD-O code: 9492/0
Line 302: Line 321:
* Non-neoplastic, reticulin-rich glial stroma.
* Non-neoplastic, reticulin-rich glial stroma.


==Ganglioglioma==
===Ganglioglioma===
:'''Not''' to be confused with ''[[ganglioneuroma]]''.
:'''Not''' to be confused with ''[[ganglioneuroma]]''.
===General===
====General====
*Grade I WHO mixed neuronal-glial tumour.
*Grade I WHO mixed neuronal-glial tumour.
*ICD-O code: 9505/1 (Anaplastic ganglioglioma: 9505/3)
*ICD-O code: 9505/1 (Anaplastic ganglioglioma: 9505/3)
Line 311: Line 330:
*Recognized as a cause of [[epilepsy]].<ref name=pmid12125968>{{Cite journal  | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi =  | PMID = 12125968 }}</ref>
*Recognized as a cause of [[epilepsy]].<ref name=pmid12125968>{{Cite journal  | last1 = Im | first1 = SH. | last2 = Chung | first2 = CK. | last3 = Cho | first3 = BK. | last4 = Lee | first4 = SK. | title = Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. | journal = J Neurooncol | volume = 57 | issue = 1 | pages = 59-66 | month = Mar | year = 2002 | doi =  | PMID = 12125968 }}</ref>


===Microscopic===
====Microscopic====
Features:
Features:
*Dysplastic neurons.
*Dysplastic neurons.
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*Necrosis
*Necrosis


===IHC===
====IHC====
*Neurons:
*Neurons:
**[[MAP2]] +ve
**[[MAP2]] +ve
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**CD34+/-ve
**CD34+/-ve


===DDx:===
====DDx:====
*[[DNT]].
*[[DNT]].
*[[Oligodendroglioma]].
*[[Oligodendroglioma]].
*Trapped cortical neurons in diffuse astrocytoma.
*Trapped cortical neurons in diffuse astrocytoma.


===Images===
====Images====
<gallery>
<gallery>
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian)
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian)
Line 347: Line 366:
*[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)].
*[http://path.upmc.edu/cases/case282.html Ganglioglioma - case 2 (upmc.edu)].


==Dysembryoplastic neuroepithelial tumour==
*Abbreviated ''DNT''.
{{Main|Dysembryoplastic neuroepithelial tumour}}


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


==Atypical teratoid/rhabdoid tumour==
==Pineal tumours==
:See also: ''[[Extrarenal malignant rhabdoid tumour]]''.
{{Main|Pineal gland}}
*Commonly abbreviated ''AT/RT''.
*May be written ''atypical teratoid rhabdoid tumour'', i.e. without the forward slash, or ''atypical teratoid-rhabdoid tumour'' (AT-RT).
{{Main|Atypical teratoid/rhabdoid tumour}}


==Medulloblastoma==
* [[Pineocytoma]]
{{Main|Medulloblastoma}}
* [[Pineal parenchymal tumour of intermediate differentiation]]
*Tumour of cerebellum - key feature.
* [[Pineoblastoma]]
*Morphologically identical supratentorial tumours are called [[primitive neuroectodermal tumour]] (PNET).
* [[Papillary tumour of the pineal region]]


==Primitive neuroectodermal tumour==  
==Embryonal tumours==
{{Main|Primitive neuroectodermal tumour}}
* [[Atypical teratoid/rhabdoid tumour]] (AT/RT) or (AT-RT)
 
* [[Medulloblastoma]]
==Embryonal tumour with abundant neuropil and true rosettes==
* [[Primitive neuroectodermal tumour]] (PNET)
*Abbreviated ''ETANTR''.
* [[Embryonal tumour with abundant neuropil and true rosettes]] (ETANTR)
{{Main|Embryonal tumour with abundant neuropil and true rosettes}}
 
==Astroblastoma==
*No WHO grade yet.
*Very rare superficial tumor of young age.
*Large, cystic. Pushing margin towards CNS.
*Vasocentric growth, plump cells with absence of fibrillary pattern.
*GFAP+ve, Synaptohysin-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.
 
<gallery>
File:Astroblastoma_HE_Specimen.jpg | HE. (WC/jensflorian)
File:Astroblastoma_HE_papillae.jpg | HE. (WC/jensflorian)
File:Astroblastoma.jpg | Astroblastoma (AFIP)
</gallery>
 
 
==Meningioma==
{{Main|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}}
 
==Neurofibroma==
{{Main|Neurofibroma}}


==Ganglioneuroma==
===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>
{{Main|Ganglioneuroma}}
{{Main|Ganglioneuroma}}
==Meningioma==
{{Main|Meningioma}}


==Chordoma==
==Chordoma==
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*Bcl-6 +ve.
*Bcl-6 +ve.
*Bcl-1 -ve.
*Bcl-1 -ve.
==Neurocytoma==
{{Main|Neurocytoma}}
==Central neurocytoma==
*Abbreviated ''CNC''.
{{Main|Central neurocytoma}}




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


==Ganglioneuroblastoma==
==Ganglioneuroblastoma==

Revision as of 13:41, 14 October 2015

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.

There are separate articles for peripheral nerve sheath tumours and pituitary/peri-pituitary lesions.

Brain tumours - overview

Alphabetical

For overview see here

By age group

Adult

Four most common types of brain tumours:[1]

  1. Metastatic brain tumours (barely edges out primary tumours)
  2. Glioblastoma (previously known as glioblastoma multiforme).
  3. Anaplastic astrocytoma.
  4. Meningioma.

Children

  1. Pilocytic astrocytoma.
  2. Medulloblastoma.
  3. Ependymoma.

Location (most common)

Certain tumours like to hang-out at certain places:[2]

Cerebrum

Cerebellum

Spinal cord

Filum terminale

  • Filum terminale = bottom end of the spinal cord - has a limited differential.

DDx:[3]

Cerebellopontine angle

  • Abbreviated CP angle.

DDx:[4]

Cystic tumours

DDx:[5]

Primary versus secondary

  • AKA (primary) brain tumour versus metastatic cancer.

Primary

Glial tumours:

  • Cytoplasmic processes - key feature.
    • Best seen at highest magnification - usu. ~1 micrometer.
    • Processes may branch.
  • Ill-defined border/blend with the surrounding brain.

Lymphoma:

  • Large (lymphoid) cells, ergo usu. not a difficult diagnosis.
    • ~2x size of resting lymphocyte, nucleoli.
  • Lesion predominantly perivascular.

Secondary

Carcinomas:

  • Well-demarcated border between brain and lesion - key feature.
  • No cytoplasmic processes.
  • Usu. have nuclear atypia of malignancy.
    • Nuclei often ~3-4x the size of a RBC.
  • +/-Glandular arrangement.
  • +/-Nucleoli.

Common neuropathology tumours in a table

Type Key feature(s) Imaging History Notes IHC Images
Normal tissue cells regularly spaced, no nuc. atypia small lesion? / deep lesion? variable missed lesion? nil
Normal. (WC)
Reactive astrocytes astrocytes with well-demarcated eosinophilic cytoplasm, regular spacing, no nuc. atypia small lesion? / deep lesion? variable missed lesion / close to a lesion; non-specific pathologic process - need more tissue nil
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
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
Meningioma. (WC)
Infiltrative astrocytoma (WHO grade II or grade III) 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+/-, GFAP+
High-grade astrocytoma. (WC)
Glioblastoma (WHO grade IV) 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 usu. old, occ. young very common, esp. glioblastoma IDH-1+/-, GFAP+
Glioblastoma. (WC)
Metastasis sharp interface with brain, often glandular, +/-nucleoli, no glial processes often cerebellular, well-circumscribed usu. old often suspected to have metastatic disease TTF-1, CK7, CK20, BRST-2
Metastasis. (WC)

† Rosenthal fibres at the periphery of a lesion are a non-specific finding seen in chronic processes.

Brain metastasis

Infiltrative astrocytomas

Overview

Notes:

Microscopic

Features:[7][8]

  • Glial processes - key feature.
    • Thin stringy cytoplasmic processes - best seen at high power in less cellular areas.
  • No Rosenthal fibres within the tumour itself.

Images:

Notes:

  • Glial vs. non-glial tumours:
    • Glial: "blends into brain"/gradual transition to non-tumour brain.
    • Non-glial: no glial processes.
  • Rosenthal fibres within the tumour... make it into a 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.[9]

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.

Images

Glioblastoma:

Anaplastic astrocytoma:

Table of common gliomas - grading

Histomorphologic comparison of common gliomas:

Entity Rosenthal
fibres / EGBs
Nuclear atypia Mitoses Necrosis or MVP Infiltrative Image
Pilocytic astrocytoma yes usu. no usu. no usu. no no
Rosenthal HE 40x.jpg
Low-grade astrocytoma no yes no no yes
Astrocytoma whoII HE.jpg
Anaplastic astrocytoma no yes yes no yes
Anaplastic astrocytoma - high mag.jpg
Glioblastoma no yes yes yes yes
Glioblastoma - high mag.jpg

Notes:

  • MVP = microvascular proliferation.
  • EGBs = eosinophilic granular bodies.

IHC

  • GFAP - should stain cytoplasm of tumour cells and the perikaryon (nuclear membrane).
  • Ki-67 - usu. high >20% of cells.
  • p53 - often +ve.
  • IDH1 (isocitrate dehydrogenase 1).

Notes:

  • IDH1 and IDH2 mutations - better survival.[11]

Astrocytic tumours

Oligodendroglial tumours

Ependymal tumours

Choroid plexus tumours

Other neuroepithelial tumours

Astroblastoma

  • No WHO grade yet.
  • Very rare superficial tumor of young age.
  • Large, cystic. Pushing margin towards CNS.
  • Vasocentric growth, plump cells with absence of fibrillary pattern.
  • GFAP+ve, Synaptohysin-ve, focally EMA/panCK+ve. MIB-1: 1-18 %.

Chordoid glioma of the third ventricle

  • WHO grade II.
  • Slowly growing, non-invasive.
  • Clusters of epithelioid cells in mucinous stroma.
  • Lymphocytic infiltrates, adjacent Rosenthal fibers.
  • Few mitoses.
  • GFAP+ve, MIB-1 1-3%.

Neuronal and mixed neuronal/glial tumours

Gangliocytoma

  • Grade I WHO neuronal tumour.
    • ICD-O code: 9492/0
  • Groups of irregular large neurons.
  • Non-neoplastic, reticulin-rich glial stroma.

Ganglioglioma

Not to be confused with ganglioneuroma.

General

  • Grade I WHO mixed neuronal-glial tumour.
  • ICD-O code: 9505/1 (Anaplastic ganglioglioma: 9505/3)
  • Rare.
  • Usu. temporal lobe.
  • Recognized as a cause of epilepsy.[12]

Microscopic

Features:

  • Dysplastic neurons.
    • Out of regular architecture / abnormal location.
    • Cytomegaly
    • Clustering
    • Binucleated (very occassionally).
  • Atypical glia.
  • Calcification.
  • Lymphocytic cuffing.

Anaplastic ganglioglioma:

  • Brisk mitotic activity
  • Necrosis

IHC

  • Neurons:
    • MAP2 +ve
    • Synaptophysin +ve
    • Neurofilament +ve
  • Glia:
    • CD34+/-ve

DDx:

Images


Lhermitte-Duclos disease

  • Abbreviated LDD.
  • AKA dysplastic cerebellar gangliocytoma.[13]
  • AKA dysplastic gangliocytoma of the cerebellum.

Pineal tumours

Embryonal tumours

Peripheral nerve sheath tumours

A classification:[14] Benign:

Malignant:

Ganglioneuroma

Not to be confused with ganglioglioma.


Meningioma

Chordoma

Hemangioblastoma

CNS lymphoma

Classification:

  • Primary CNS lymphoma.
  • Non-primary CNS lymphoma - see lymphoma article.

General - primary CNS

  • Classically periventicular distribution.
  • Usually large B cell; can be considered a type of diffuse large B cell lymphoma (DLBCL).
    • Prognosis of CNS (DLBCL) lymphomas worse than nodal (non-CNS) DLBCL.[16]

Microscopic

Features:

  • Large cell lymphoma.
    • Size = 2x diameter normal lymphocyte.
    • Nucleolus - common.
  • Perivascular clustering.

Images

www:

IHC

Can be subclassified in GCB (germinal centre B-cell-like) and non-GCB by CD10, Bcl-6, MUM1/IRF-4, and Bcl-2.[16]

Common pattern:

  • CD20 +ve - key stain.
  • CD3 -ve.
  • Ki-67 ~40%.
  • Bcl-6 +ve.
  • Bcl-1 -ve.


Ganglioneuroblastoma

General

  • Uncommon.
  • Part of the neuroblastic tumours group which includes:[17]

Microscopic

Features:

  • Ganglion-like cells with a prominent nucleolus.
  • Small undifferentiated cells with scant cytoplasm.

Images:

IHC

  • NSE +ve -- small cells.

Lesions of the sella turcica

Lesions of the sella turcica, the pituitary gland environs, is a topic for it self. The differential diagnosis for lesions in this area includes:

See also

References

  1. http://neurosurgery.mgh.harvard.edu/abta/primer.htm
  2. URL: http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/files/4ce563fb7e8e48fc9ed8b42e296a7747.gif and http://www.msdlatinamerica.com/ebooks/DiagnosticNeuropathologySmears/sid117213.html. Accessed on: 2 November 2010.
  3. JLK. 31 May 2010.
  4. R. Kiehl. 8 November 2010.
  5. URL: http://path.upmc.edu/cases/case320/dx.html. Accessed on: 14 January 2012.
  6. URL: http://www.pathologyoutlines.com/Cnstumor.html#cystsgeneral. Accessed on: 14 January 2012.
  7. Rong Y, Durden DL, Van Meir EG, Brat DJ (June 2006). "'Pseudopalisading' necrosis in glioblastoma: a familiar morphologic feature that links vascular pathology, hypoxia, and angiogenesis". J. Neuropathol. Exp. Neurol. 65 (6): 529–39. PMID 16783163.
  8. http://dictionary.reference.com/browse/palisading
  9. URL: http://path.upmc.edu/cases/case79/dx.html. Accessed on: 2 January 2012.
  10. Yan H, Parsons DW, Jin G, et al. (February 2009). "IDH1 and IDH2 mutations in gliomas". N. Engl. J. Med. 360 (8): 765–73. doi:10.1056/NEJMoa0808710. PMC 2820383. PMID 19228619. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2820383/.
  11. Houillier C, Wang X, Kaloshi G, et al. (October 2010). "IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas". Neurology 75 (17): 1560–6. doi:10.1212/WNL.0b013e3181f96282. PMID 20975057.
  12. Im, SH.; Chung, CK.; Cho, BK.; Lee, SK. (Mar 2002). "Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome.". J Neurooncol 57 (1): 59-66. PMID 12125968.
  13. Yağci-Küpeli, B.; Oguz, KK.; Bilen, MA.; Yalçin, B.; Akalan, N.; Büyükpamukçu, M. (Mar 2010). "An unusual cause of posterior fossa mass: Lhermitte-Duclos disease.". J Neurol Sci 290 (1-2): 138-41. doi:10.1016/j.jns.2009.12.010. PMID 20060133.
  14. Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A (October 2007). "Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns". AJNR Am J Neuroradiol 28 (9): 1633–8. doi:10.3174/ajnr.A0682. PMID 17893219. http://www.ajnr.org/cgi/reprint/28/9/1633.
  15. URL: http://medical-dictionary.thefreedictionary.com/ganglioma. Accessed on: 8 November 2010.
  16. 16.0 16.1 Raoux D, Duband S, Forest F, et al. (June 2010). "Primary central nervous system lymphoma: Immunohistochemical profile and prognostic significance". Neuropathology 30 (3): 232–40. doi:10.1111/j.1440-1789.2009.01074.x. PMID 19925562.
  17. Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B (July 1999). "Terminology and morphologic criteria of neuroblastic tumors: recommendations by the International Neuroblastoma Pathology Committee". Cancer 86 (2): 349–63. PMID 10421272.

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