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An '''astrocytoma''' is a neoplasm derived from an [[neurohistology|astrocyte]]. Astrocytomas are common. | 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 | ||
|- | |- | ||
| | | Astrocytoma, IDH mutant WHO CNS grade 2 | ||
| diffuse | | diffuse | ||
| protoplasmatic, fibrillar | | adults | ||
| Diffuse, protoplasmatic, fibrillar or gemistocytic astrocytoma. | |||
| [[File:Astrocytoma whoII HE.jpg|thumb|center|150px]] | | [[File:Astrocytoma whoII HE.jpg|thumb|center|150px]] | ||
|- | |- | ||
| | | 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 | | 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 | | 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 | | Pleomorphic xanthoastrocytoma, WHO CNS grade 2 (PXA) | ||
| circumscribed | | circumscribed | ||
| | | young adults | ||
| | |||
| [[File:PXA_HE_x20.jpg|thumb|center|150px]] | | [[File:PXA_HE_x20.jpg|thumb|center|150px]] | ||
|- | |- | ||
| Subependymal giant cell astrocytoma, WHO | | 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 | | 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}} | ||
== | ==Astrocytoma, IDH mutant== | ||
* Grade II | * Astrocytoma, IDH mutant are less common than glioblastoma. | ||
* Usually | * 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=== | ||
* Grade III | * 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. | ||
===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. | ||
* | * 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== | ||
* | * Depreceated entity. | ||
* 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. | ||
==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= |