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Astrocytoma (view source)

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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}}


==Anaplastic astrocytoma==
=== Astrocytoma, IDH mutant grade 2===
* Grade III astrocytic tumors typically seen in adults.
* 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.
* 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==
* Extensively diffusely growing astrocytic neoplasm.
* Depreceated entity.
**Currently considered a rare pattern of diffuse glioma infiltration.
* Was used for extensively diffusely growing astrocytic neoplasms.
**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>
**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).
* More than 3 lobes have to be involved, us. bilateral (radiology required).
* biologic behaviour corresponds to WHO III (ICD-O: 9381/3)
* biologic behaviour corresponds to WHO III (ICD-O: 9381/3)
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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.
** It is likely that suggests that in the upcoming WHO classification gliomatosis is no longer a separate glioma entity.<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>
==H3.3 K27M mutated glioma of the midline==
* High-grade astrocytic neoplasm associated with midline structures
* Mostly in children and adolescents
* Includes diffuse intrinsic pontine gliomas (DPIG)
* Will become provisonal variant in upcoming WHO 2016 classification
* 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>
<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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*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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* Usually not dura-based (DD: Desmoplastic infantile astrocytoma)
* Usually not dura-based (DD: Desmoplastic infantile astrocytoma)
* Glial tumor with non-neoplastic fibromatous component.
* Glial tumor with non-neoplastic fibromatous component.


=See also=
=See also=
Jensflorian
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