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An '''astrocytoma''' is a neoplasm derived from an [[neurohistology|astrocyte]].  Astrocytomas are common.  This article is a brief introduction them. 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.
 
=Categorization=
Astrocytomas can be categorized in serveral ways.
* Common vs. uncommon tumours.
* Adult vs. pediatric tumours.
* Circumscribed vs. diffusely growing astrocytomas.
 
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=
=Overview=
These astrocytic tumors are frequently diagnosed in neuropathology practice:
{| class="wikitable sortable"  
{| class="wikitable sortable"  
! Name
! Name
! Type
! Type
! Variants / Patterns
! Age
! Variants / Patterns / Other designations
! Image
! Image
|-
|-
| Diffuse Astrocytoma, WHO II
| Astrocytoma, IDH mutant WHO CNS grade 2
| diffuse
| diffuse
| protoplasmatic, fibrillar, gemistocytic
| adults
| Diffuse, protoplasmatic, fibrillar or gemistocytic astrocytoma.
| [[File:Astrocytoma whoII HE.jpg|thumb|center|150px]]
| [[File:Astrocytoma whoII HE.jpg|thumb|center|150px]]
|-
|-
| Anaplastic Astrocytoma, WHO III
| Astrocytoma, IDH mutant WHO CNS grade 3
| diffuse
| diffuse
| gliomatosis cerebri
| adults
| Anaplastic astrocytoma, gliomatosis cerebri
| [[File:Anaplastic_astrocytoma_-_very_high_mag.jpg|thumb|center|150px]]
| [[File:Anaplastic_astrocytoma_-_very_high_mag.jpg|thumb|center|150px]]
|-
|-
| Glioblastoma, WHO IV
| Astrocytoma, IDH mutant WHO CNS grade 4
| diffuse
| adults
|
| [[File:IDH1_GBM_20x.jpg|thumb|center|150px]]
|-
| Glioblastoma, WHO CNS grade 4
| diffuse
| diffuse
| adults
| small cell, epitheloid/rhabdoid, with PNET componet, with granular cell component, giant cell, gliosarcoma
| small cell, epitheloid/rhabdoid, with PNET componet, with granular cell component, giant cell, gliosarcoma
| [[File:Glioblastoma_(1).jpg|thumb|center|150px]]
| [[File:Glioblastoma_(1).jpg|thumb|center|150px]]
|-
|-
| Pilocytic astrocytoma, WHO I
| 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
| circumscribed
| children
| pilomyxoid astrocytoma, anaplastic pilocytic astrocytoma
| pilomyxoid astrocytoma, anaplastic pilocytic astrocytoma
| [[File:Rosenthal_HE_40x.jpg|thumb|center|150px]]
| [[File:Rosenthal_HE_40x.jpg|thumb|center|150px]]
|-
|-
| Pleomorphic xanthoastrocytoma, WHO II (PXA)
| Pleomorphic xanthoastrocytoma, WHO CNS grade 2 (PXA)
| circumscribed
| circumscribed
| anaplastic PXA
| young adults
|
| [[File:PXA_HE_x20.jpg|thumb|center|150px]]
| [[File:PXA_HE_x20.jpg|thumb|center|150px]]
|-
|-
| Subependymal giant cell astrocytoma, WHO I (SEGA)
| Pleomorphic xanthoastrocytoma, WHO CNS grade 3 (PXA)
| circumscribed
| 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
| SEGA in tuberous sclerosis
| [[File:SEGA_HE.jpg|thumb|center|150px]]
| [[File:SEGA_HE.jpg|thumb|center|150px]]
|}
|}
=Common=
 
=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==
==Pilocytic astrocytoma==
* Benign, cystic, infratentorial.
* Benign, cystic, infratentorial.
* Classic childhood tumor, surgically resectable.
* Classic childhood tumor, surgically resectable.
* Circumscribed astrocytic glioma
* Variant: [[Pilomyxoid astrocytoma]]
* Variant: [[Pilomyxoid astrocytoma]]
{{Main|Pilocytic astrocytoma}}
{{Main|Pilocytic astrocytoma}}


==Diffuse astrocytoma==
==Astrocytoma, IDH mutant==
* Grade II astrocytic tumors typically seen in adults.
* Astrocytoma, IDH mutant are less common than glioblastoma.
* Usually show progression to glioblastoma.
* Grade 2-4 depends on histological and molecular criteria:
{{Main|Diffuse astrocytoma}}
{{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.


==Anaplastic astrocytoma==
===Astrocytoma, IDH mutant grade 3===
* Grade III astrocytic tumors typically seen in adults.
* Formerly designated Anaplastic astrocytoma Grade III.
* Typically seen in adults.
* Increased cellularity, cell atypia and mitotic activity.
* Lacks endothelial proliferations and necrosis of glioblastoma.
* Lacks endothelial proliferations and necrosis of glioblastoma.
{{Main|Anaplastic astrocytoma}}
 
===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==
==Glioblastoma==
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{{Main|Glioblastoma}}
{{Main|Glioblastoma}}


=Uncommon=
=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==
==Subependymal giant cell astrocytoma==
* Intraventricular benign tumor of adolescents.
* Intraventricular benign tumor of adolescents.
* Assoicated with [[Tuberous sclerosis]].
* Associated with [[Tuberous sclerosis]].
{{Main|Subependymal giant cell astrocytoma}}
{{Main|Subependymal giant cell astrocytoma}}


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* Large lipidized cells mimicking a malignant tumor  
* Large lipidized cells mimicking a malignant tumor  
{{Main|Pleomorphic xanthoastrocytoma}}
{{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}}
==Diffuse hemispheric glioma, H3 G34-mutant==
* Infiltrative hemispheric glioma of young adults.
* Glioblastoma-like appearance (CNS WHO grade 4 tumor).
* Newly defined entity in WHO 2021 classification.
* H3F3A missense mutation G34R or G34V.
{{Main|Diffuse hemispheric glioma, H3 G34-mutant}}
==High-grade astrocytoma with piloid features==
* Frequent in posterior fossa (75%).
* 1-3% of all brain tumors.
* 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}}


==Gliomatosis cerebri==
==Gliomatosis cerebri==
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** GC type 2: with a solid portion, mostly IDH1 mutant.
** GC type 2: with a solid portion, mostly IDH1 mutant.
* Genetic studies indicate strong overlap with diffuse astrocytic gliomas, oligodendrogliomas and glioblastoma.
* Genetic studies indicate strong overlap with diffuse astrocytic gliomas, oligodendrogliomas and glioblastoma.
==Diffuse midline glioma, H3 K27M mutant==
* High-grade astrocytic neoplasm associated with midline structures (thalamus, brain stem, spinal cord).
* Mostly in children and adolescents.
* Includes diffuse intrinsic pontine gliomas (DPIG).
* Newly defined entity since WHO 2016 classification.<ref>{{Cite journal  | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref>
* Distinct biological and clinical group with poor prognosis.<ref>{{Cite journal  | last1 = Khuong-Quang | first1 = DA. | last2 = Buczkowicz | first2 = P. | last3 = Rakopoulos | first3 = P. | last4 = Liu | first4 = XY. | last5 = Fontebasso | first5 = AM. | last6 = Bouffet | first6 = E. | last7 = Bartels | first7 = U. | last8 = Albrecht | first8 = S. | last9 = Schwartzentruber | first9 = J. | title = K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. | journal = Acta Neuropathol | volume = 124 | issue = 3 | pages = 439-47 | month = Sep | year = 2012 | doi = 10.1007/s00401-012-0998-0 | PMID = 22661320 }}</ref>
** EGFR amplification is usu. absent.<ref>{{Cite journal  | last1 = Meyronet | first1 = D. | last2 = Esteban-Mader | first2 = M. | last3 = Bonnet | first3 = C. | last4 = Joly | first4 = MO. | last5 = Uro-Coste | first5 = E. | last6 = Amiel-Benouaich | first6 = A. | last7 = Forest | first7 = F. | last8 = Rousselot-Denis | first8 = C. | last9 = Burel-Vandenbos | first9 = F. | title = Characteristics of H3 K27M-mutant gliomas in adults. | journal = Neuro Oncol | volume = 19 | issue = 8 | pages = 1127-1134 | month = Aug | year = 2017 | doi = 10.1093/neuonc/now274 | PMID = 28201752 }}</ref>
** Tumors usu. have unmethylated MGMT promotor.<ref>{{Cite journal  | last1 = Banan | first1 = R. | last2 = Christians | first2 = A. | last3 = Bartels | first3 = S. | last4 = Lehmann | first4 = U. | last5 = Hartmann | first5 = C. | title = Absence of MGMT promoter methylation in diffuse midline glioma, H3 K27M-mutant. | journal = Acta Neuropathol Commun | volume = 5 | issue = 1 | pages = 98 | month = 12 | year = 2017 | doi = 10.1186/s40478-017-0500-2 | PMID = 29246238 }}</ref>
* MRI: May be or be not enhancing.
*Histologic spectrum ranges from minimal hypercellularity to full-blown glioblastoma.
Note: Cases may also appear outside midline structures and in adult patients.<ref>{{Cite journal  | last1 = Nakata | first1 = S. | last2 = Nobusawa | first2 = S. | last3 = Yamazaki | first3 = T. | last4 = Osawa | first4 = T. | last5 = Horiguchi | first5 = K. | last6 = Hashiba | first6 = Y. | last7 = Yaoita | first7 = H. | last8 = Matsumura | first8 = N. | last9 = Ikota | first9 = H. | title = Histone H3 K27M mutations in adult cerebellar high-grade gliomas. | journal = Brain Tumor Pathol | volume = 34 | issue = 3 | pages = 113-119 | month = Jul | year = 2017 | doi = 10.1007/s10014-017-0288-6 | PMID = 28547652 }}</ref>
<gallery>
File:K27M mutant diffuse glioma of the midline.jpg|Nuclear [[H3F3A]] K27M immunostaining in a diffuse glioma of the midline. (WC/jensflorian)
</gallery>


==Gliosarcoma==
==Gliosarcoma==
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