Difference between revisions of "Astrocytoma"

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An '''astrocytoma''' is a neoplasm derived from an [[neurohistology|astrocyte]].  Diffuse astrocytomas are common 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 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=
Until 2016 WHO classification, roman numerals I-IV were used for grading. The upcoming 2021 WHO classification will use arabic numbering 1-4 instead.
These astrocytic tumors are frequently diagnosed in neuropathology practice:
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=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.
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* Astrocytoma, IDH mutant are less common than glioblastoma.
* Astrocytoma, IDH mutant are less common than glioblastoma.
* Grade 2-4 depends on histological and molecular criteria:
* Grade 2-4 depends on histological and molecular criteria:
{{Main|Astrocytoma, IDH-mutant}}


=== Astrocytoma, IDH mutant grade 2===
=== Astrocytoma, IDH mutant grade 2===
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* Typically seen in adults.
* Typically seen in adults.
* Usually shows progression to astrocytoma IDH mutant, grade 4.
* Usually shows progression to astrocytoma IDH mutant, grade 4.
{{Main|Diffuse astrocytoma}}


===Astrocytoma, IDH mutant grade 3===
===Astrocytoma, IDH mutant grade 3===
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* Increased cellularity, cell atypia and mitotic activity.
* 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===
===Astrocytoma, IDH mutant grade 4===
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{{Main|Glioblastoma}}
{{Main|Glioblastoma}}


=Uncommon=
=Uncommon Astrocytomas=
==Diffuse astrocytoma, MYB- or MYBL-altered==
==Diffuse astrocytoma, MYB- or MYBL-altered==
* Pediatric-type diffuse low-grade glioma.
* Pediatric-type diffuse low-grade glioma.
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* Large lipidized cells mimicking a malignant tumor  
* Large lipidized cells mimicking a malignant tumor  
{{Main|Pleomorphic xanthoastrocytoma}}
{{Main|Pleomorphic xanthoastrocytoma}}
==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>
**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.


==Diffuse midline glioma, H3 K27-altered==
==Diffuse midline glioma, H3 K27-altered==
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* Mostly in children and adolescents.
* Mostly in children and adolescents.
* Includes diffuse intrinsic pontine gliomas (DPIG).
* 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>
{{Main|Diffuse midline glioma, H3 K27-altered}}
File:K27M mutant diffuse glioma of the midline.jpg|Nuclear [[H3F3A]] K27M immunostaining in a diffuse glioma of the midline. (WC/jensflorian)
</gallery>


==Diffuse hemispheric glioma, H3 G34-mutant==
==Diffuse hemispheric glioma, H3 G34-mutant==
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* H3F3A missense mutation G34R or G34V.
* H3F3A missense mutation G34R or G34V.
{{Main|Diffuse hemispheric glioma, H3 G34-mutant}}
{{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==
* 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>
**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.


==Gliosarcoma==
==Gliosarcoma==
Jensflorian
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