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An '''astrocytoma''' is a neoplasm derived from an [[neurohistology|astrocyte]].  Astrocytomas are common.  This article deals with, among other things, rare astrocytomas. An overview of CNS tumours is found in the ''[[CNS tumours]]'' article.
An '''astrocytoma''' is a neoplasm thought to be derived from an [[neurohistology|astrocyte]].  Astrocytomas/Glioblastomas are most common type of glial tumours and grouped together with [[Oligodendroglioma]] and glioneuronal tumours in the current WHO brain tumor classficiation.  Some (often circumscribed) astrocytic tumors and pediatric tumours are biologically different from adult-onset diffuse astrocytomas. An overview of other CNS tumours is found in the ''[[CNS tumours]]'' article.


=Other=
=Categorization=
==Pleomorphic xanthroastrocytoma==
Astrocytomas can be categorized in serveral ways.
*Abbreviated ''PXA''.
* Common vs. uncommon tumours.
===General===
* Adult vs. pediatric tumours.
*Kids & young adults.
* Circumscribed vs. diffusely growing astrocytomas.
*Prognosis usu. good.
 
Until 2016 WHO classification, roman numerals I-IV were used for grading. The current 2021 WHO classification uses arabic numbering 1-4 for CNS WHO grading instead.
 
=Overview=
These astrocytic tumors are frequently diagnosed in neuropathology practice:
{| class="wikitable sortable"
! Name
! Type
! Age
! Variants / Patterns / Other designations
! Image
|-
| Astrocytoma, IDH mutant WHO CNS grade 2
| diffuse
| adults
| Diffuse, protoplasmatic, fibrillar or gemistocytic astrocytoma.
| [[File:Astrocytoma whoII HE.jpg|thumb|center|150px]]
|-
| Astrocytoma, IDH mutant WHO CNS grade 3
| diffuse
| adults
| Anaplastic astrocytoma, gliomatosis cerebri
| [[File:Anaplastic_astrocytoma_-_very_high_mag.jpg|thumb|center|150px]]
|-
| Astrocytoma, IDH mutant WHO CNS grade 4
| diffuse
| adults
|
| [[File:IDH1_GBM_20x.jpg|thumb|center|150px]]
|-
| Glioblastoma, WHO CNS grade 4
| diffuse
| adults
| small cell, epitheloid/rhabdoid, with PNET componet, with granular cell component, giant cell, gliosarcoma
| [[File:Glioblastoma_(1).jpg|thumb|center|150px]]
|-
| Diffuse midline glioma, H3 K27M-mutant, WHO CNS grade 4
| diffuse
| children
|
| [[File:K27M mutant diffuse glioma of the midline.jpg|thumb|center|150px]]
|-
|-
| Pilocytic astrocytoma, WHO CNS grade 1
| circumscribed
| children
| pilomyxoid astrocytoma, anaplastic pilocytic astrocytoma
| [[File:Rosenthal_HE_40x.jpg|thumb|center|150px]]
|-
| Pleomorphic xanthoastrocytoma, WHO CNS grade 2 (PXA)
| circumscribed
| young adults
|
| [[File:PXA_HE_x20.jpg|thumb|center|150px]]
|-
| Pleomorphic xanthoastrocytoma, WHO CNS grade 3 (PXA)
| circumscribed
| young adults
| Anaplastic PXA.
| [[File:Anaplastic_pxa_histology.jpg|thumb|center|150px]]
|-
| Subependymal giant cell astrocytoma, WHO CNS grade 1 (SEGA)
| circumscribed
| young adults
| SEGA in tuberous sclerosis
| [[File:SEGA_HE.jpg|thumb|center|150px]]
|}
 
=Adult-type astrocytomas=
*[[Astrocytoma, IDH-mutant]].
*[[Glioblastoma]], IDH wildtype.
*[[High-grade astrocytoma with piloid features]].
*[[Pleomorphic xanthroastrocytoma]].
*[[Subependymal giant cell astrocytoma]].
*Chordoid glioma.
 
=Pediatric-type astrocytomas=
*[[Pilocytic astrocytoma]].
*[[Pediatric-type diffuse high-grade glioma]].
*[[Pediatric-type diffuse low-grade glioma]].
*[[Astroblastoma]], MN1-altered.
 
=Diffuse growing astrocytomas=
*[[Astrocytoma, IDH-mutant]].
*[[Glioblastoma]], IDH wildtype.
*[[Diffuse midline glioma, H3 K27-altered]].
*[[Diffuse hemispheric glioma, H3 G34-mutant]].
*[[Diffuse astrocytoma, MYB- or MYBL-altered]].
*Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype.
*Angiocentric glioma.
*Diffuse low-grade glioma, MAPK pathway-altered.
 
=Circumscribed astrocytomas=
*[[Pilocytic astrocytoma]].
*[[High-grade astrocytoma with piloid features]].
*[[Pleomorphic xanthroastrocytoma]].
*[[Subependymal giant cell astrocytoma]].
*[[Chordoid glioma]].
*[[Astroblastoma]], MN1-altered.
 
=Common Astrocytomas=
==Pilocytic astrocytoma==
* Benign, cystic, infratentorial.
* Classic childhood tumor, surgically resectable.
* Circumscribed astrocytic glioma
* Variant: [[Pilomyxoid astrocytoma]]
{{Main|Pilocytic astrocytoma}}
 
==Astrocytoma, IDH mutant==
* Astrocytoma, IDH mutant are less common than glioblastoma.
* Grade 2-4 depends on histological and molecular criteria:
{{Main|Astrocytoma, IDH-mutant}}
 
=== Astrocytoma, IDH mutant grade 2===
* Formerly designated as Diffuse astrocytoma Grade II.
* Typically seen in adults.
* Usually shows progression to astrocytoma IDH mutant, grade 4.
 
===Astrocytoma, IDH mutant grade 3===
* Formerly designated Anaplastic astrocytoma Grade III.
* Typically seen in adults.
* Increased cellularity, cell atypia and mitotic activity.
* Lacks endothelial proliferations and necrosis of glioblastoma.
 
===Astrocytoma, IDH mutant grade 4===
* Formerly called Glioblastoma, IDH mutant.
* Endothelial proliferations and necrosis  indistinguishable from glioblastoma.
* Homozygous CDKN2A deletion qualifies grade 2 and grade 3 astrocytomas as grade 4 tumor.
 
==Glioblastoma==
* Most common malignant brain tumor peaking around 65 years.
* Prognosis very poor.
* Variant: [[Giant cell glioblastoma]]
* Variant: [[Gliosarcoma]]
{{Main|Glioblastoma}}
 
=Uncommon Astrocytomas=
==Diffuse astrocytoma, MYB- or MYBL-altered==
* Pediatric-type diffuse low-grade glioma.
* Associated with epileptic seizures.
* Excellent prognosis.
{{Main|Diffuse astrocytoma, MYB- or MYBL-altered}}
 
==Subependymal giant cell astrocytoma==
* Intraventricular benign tumor of adolescents.
* Associated with [[Tuberous sclerosis]].
{{Main|Subependymal giant cell astrocytoma}}
 
==Pleomorphic xanthroastrocytoma (PXA)==
* Kids & young adults usually with good prognosis.
* Large lipidized cells mimicking a malignant tumor
{{Main|Pleomorphic xanthoastrocytoma}}
 
==Diffuse midline glioma, H3 K27-altered==
* High-grade astrocytic neoplasm associated with midline structures (thalamus, brain stem, spinal cord).
* Mostly in children and adolescents.
* Includes diffuse intrinsic pontine gliomas (DPIG).
 
{{Main|Diffuse midline glioma, H3 K27-altered}}


===Microscopic===
==Diffuse hemispheric glioma, H3 G34-mutant==
Features:
* Infiltrative hemispheric glioma of young adults.
*Large cells with intracytoplasmic lipid accumulation, i.e. foamy cytoplasm - '''key features'''.<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/neurotest/Q14-Ans.htm http://moon.ouhsc.edu/kfung/jty1/neurotest/Q14-Ans.htm]. Accessed on: 13 January 2011.</ref>
* Glioblastoma-like appearance (CNS WHO grade 4 tumor).
**May not be obvious/one may have to search for this.
* Newly defined entity in WHO 2021 classification.
*Focal marked nuclear atypia - including hyperchromasia, marked nuclear enlargement, irregular chromatin.
* H3F3A missense mutation G34R or G34V.
*Multinucleation - common.
{{Main|Diffuse hemispheric glioma, H3 G34-mutant}}


Images:
==High-grade astrocytoma with piloid features==
*[http://commons.wikimedia.org/wiki/File:Pleo_xantho.jpg PXA (WC/AFIP)].
* Frequent in posterior fossa (75%).
*[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q14-Ans.htm PXA (ouhsc.edu)].
* 1-3% of all brain tumors.
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970469-7&figureId=fig1&ddx=true PXA (pathconsultddx.com)].
* Histology may resemble Glionblastoma or Pleomorphic xanthoastrocytoma.
* Tumor is enriched for piloid cell processes.
* ATRX nuclear loss is frequent.
* Usu. MAPK-pathway alterations + CDKN2A homozygous deletion.
* Distinct methylation profile.
{{Main|High-grade astrocytoma with piloid features}}


===IHC===
==Gliomatosis cerebri==
Features:<Ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970469-7 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970469-7]. Accessed on: 13 January 2011.</ref>
* Depreceated entity.
*GFAP +ve -- required for Dx, may be patchy.
* Was used for extensively diffusely growing astrocytic neoplasms.
*S-100 +ve -- cytoplasm, usu. diffuse.
**Introduced in 1938 as a post-mortem diagnosis.<ref>SAMUEL NEVIN - GLIOMATOSIS CEREBRI, DOI: http://dx.doi.org/10.1093/brain/61.2.170 170-191 First published online: 1 June 1938</ref>
**Since 2016 it is no longer considered a distinct entity.<ref>{{Cite journal  | last1 = Johnson | first1 = DR. | last2 = Guerin | first2 = JB. | last3 = Giannini | first3 = C. | last4 = Morris | first4 = JM. | last5 = Eckel | first5 = LJ. | last6 = Kaufmann | first6 = TJ. | title = 2016 Updates to the WHO Brain Tumor Classification System: What the Radiologist Needs to Know. | journal = Radiographics | volume = 37 | issue = 7 | pages = 2164-2180 | month =  | year =  | doi = 10.1148/rg.2017170037 | PMID = 29028423 }}</ref><ref>{{Cite journal  | last1 = Herrlinger | first1 = U. | last2 = Jones | first2 = DT. | last3 = Glas | first3 = M. | last4 = Hattingen | first4 = E. | last5 = Gramatzki | first5 = D. | last6 = Stuplich | first6 = M. | last7 = Felsberg | first7 = J. | last8 = Bähr | first8 = O. | last9 = Gielen | first9 = GH. | title = Gliomatosis cerebri: no evidence for a separate brain tumor entity. | journal = Acta Neuropathol | volume =  | issue =  | pages =  | month = Oct | year = 2015 | doi = 10.1007/s00401-015-1495-z | PMID = 26493382 }}</ref>
* More than 3 lobes have to be involved, us. bilateral (radiology required).
* biologic behaviour corresponds to WHO III (ICD-O: 9381/3)
* Based on presence / absence of a solid component authors propose two types:<ref>{{Cite journal  | last1 = Seiz | first1 = M. | last2 = Tuettenberg | first2 = J. | last3 = Meyer | first3 = J. | last4 = Essig | first4 = M. | last5 = Schmieder | first5 = K. | last6 = Mawrin | first6 = C. | last7 = von Deimling | first7 = A. | last8 = Hartmann | first8 = C. | title = Detection of IDH1 mutations in gliomatosis cerebri, but only in tumors with additional solid component: evidence for molecular subtypes. | journal = Acta Neuropathol | volume = 120 | issue = 2 | pages = 261-7 | month = Aug | year = 2010 | doi = 10.1007/s00401-010-0701-2 | PMID = 20514489 }}</ref>
** GC type 1: classic diffuse growth, without IDH1/2 mutation.
** GC type 2: with a solid portion, mostly IDH1 mutant.
* Genetic studies indicate strong overlap with diffuse astrocytic gliomas, oligodendrogliomas and glioblastoma.


=Glioblastoma=
==Gliosarcoma==
==Gliosarcoma==
===General===
===General===
*Considered to be a variant of glioblastoma by WHO.<ref name=pmid19618114>{{cite journal |author=Han SJ, Yang I, Tihan T, Prados MD, Parsa AT |title=Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity |journal=J. Neurooncol. |volume=96 |issue=3 |pages=313–20 |year=2010 |month=February |pmid=19618114 |pmc=2808523 |doi=10.1007/s11060-009-9973-6 |url=}}</ref>
*Considered to be a variant of [[glioblastoma]] by WHO.<ref name=pmid19618114>{{cite journal |author=Han SJ, Yang I, Tihan T, Prados MD, Parsa AT |title=Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity |journal=J. Neurooncol. |volume=96 |issue=3 |pages=313–20 |year=2010 |month=February |pmid=19618114 |pmc=2808523 |doi=10.1007/s11060-009-9973-6 |url=}}</ref>
*Rare ~ 200 cases reported in the literature.<ref name=pmid19618114/>
*Rare ~ 200 cases reported in the literature.<ref name=pmid19618114/>
*Definition: gliosarcoma = glioblastoma + sarcomatous component.<ref name=pmid20415184>{{cite journal |author=Ayadi L, Charfi S, Khabir A, ''et al.'' |title=[Cerebral gliosarcoma: clinico-pathologic study of 8 cases] |language=French |journal=Tunis Med |volume=88 |issue=3 |pages=142–6 |year=2010 |month=March |pmid=20415184 |doi= |url=}}</ref>
*Definition: gliosarcoma = glioblastoma + sarcomatous component.<ref name=pmid20415184>{{cite journal |author=Ayadi L, Charfi S, Khabir A, ''et al.'' |title=[Cerebral gliosarcoma: clinico-pathologic study of 8 cases] |language=French |journal=Tunis Med |volume=88 |issue=3 |pages=142–6 |year=2010 |month=March |pmid=20415184 |doi= |url=}}</ref>
*Usual location (like glioblastoma): temporal lobe.
*Usual location (like glioblastoma): temporal lobe.
*Prognosis is similiar to [[glioblastoma]].<ref>{{Cite journal  | last1 = Frandsen | first1 = J. | last2 = Orton | first2 = A. | last3 = Jensen | first3 = R. | last4 = Colman | first4 = H. | last5 = Cohen | first5 = AL. | last6 = Tward | first6 = J. | last7 = Shrieve | first7 = DC. | last8 = Suneja | first8 = G. | title = Patterns of care and outcomes in gliosarcoma: an analysis of the National Cancer Database. | journal = J Neurosurg | volume =  | issue =  | pages = 1-6 | month = Jun | year = 2017 | doi = 10.3171/2016.12.JNS162291 | PMID = 28621623 }}</ref>
** Age below 65 years is prognostic.


===Microscopic===
===Microscopic===
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**Adipocyte.  
**Adipocyte.  


Image: [http://commons.wikimedia.org/wiki/File:Gliosarcoma_Histopathology_200x_EVG.jpg Gliosarcoma - elastic von Gieson (WC)].
====Images====
<gallery>
Image:Gliosarcoma_Histopathology_200x_EVG.jpg | Gliosarcoma - elastica van Giesson. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case169/micro.html Gliosarcoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case361.html Gliosarcoma - case 2 - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case367.html Gliosarcoma - case 3 - several images (upmc.edu)].
 
===IHC===
*GFAP +ve -- astrocytic component.<ref name=pmid9736406>{{Cite journal  | last1 = Horiguchi | first1 = H. | last2 = Hirose | first2 = T. | last3 = Kannuki | first3 = S. | last4 = Nagahiro | first4 = S. | last5 = Sano | first5 = T. | title = Gliosarcoma: an immunohistochemical, ultrastructural and fluorescence in situ hybridization study. | journal = Pathol Int | volume = 48 | issue = 8 | pages = 595-602 | month = Aug | year = 1998 | doi =  | PMID = 9736406 }}</ref>
**Spindle cell component -ve.<ref>URL: [http://path.upmc.edu/cases/case361.html http://path.upmc.edu/cases/case361.html]. Accessed on: 15 January 2012.</ref>
 
Gliosarcoma with smooth muscle component (gliomyosarcoma):<ref name=pmid21393877>{{Cite journal  | last1 = Khanna | first1 = M. | last2 = Siraj | first2 = F. | last3 = Chopra | first3 = P. | last4 = Bhalla | first4 = S. | last5 = Roy | first5 = S. | title = Gliosarcoma with prominent smooth muscle component (gliomyosarcoma): a report of 10 cases. | journal = Indian J Pathol Microbiol | volume = 54 | issue = 1 | pages = 51-4 | month =  | year =  | doi = 10.4103/0377-4929.77324 | PMID = 21393877 }}</ref>
*SMA +ve.
*Factor VIII +ve.
 
==Gliofibroma==
* Very rare indolent tumor in children <ref>{{Cite journal  | last1 = Deb | first1 = P. | last2 = Sarkar | first2 = C. | last3 = Garg | first3 = A. | last4 = Singh | first4 = VP. | last5 = Kale | first5 = SS. | last6 = Sharma | first6 = MC. | title = Intracranial gliofibroma mimicking a meningioma: a case report and review of literature. | journal = Clin Neurol Neurosurg | volume = 108 | issue = 2 | pages = 178-86 | month = Feb | year = 2006 | doi = 10.1016/j.clineuro.2004.11.021 | PMID = 16412839 }}</ref>
* Usually not dura-based (DD: Desmoplastic infantile astrocytoma)
* Glial tumor with non-neoplastic fibromatous component.


=See also=
=See also=
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