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Pediatric-type diffuse high-grade glioma (view source)
Revision as of 10:22, 5 April 2022
, 10:22, 5 April 2022→Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype: add last entity
Jensflorian (talk | contribs) (→Diffuse hemispheric glioma, H3 G34-mutant: fist data) |
Jensflorian (talk | contribs) (→Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype: add last entity) |
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=Diffuse midline glioma, H3 K27-altered= | |||
* High-grade astrocytic neoplasm associated with midline structures (thalamus, brain stem, spinal cord). | * High-grade astrocytic neoplasm associated with midline structures (thalamus, brain stem, spinal cord). | ||
* Mostly in children and adolescents. | * Mostly in children and adolescents. | ||
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</gallery> | </gallery> | ||
=Diffuse hemispheric glioma, H3 G34-mutant= | |||
* Diffusely infiltrating glioma in young adults and adolescents. | * Diffusely infiltrating glioma in young adults and adolescents. | ||
* WHO CNS grade 4 neoplasm. | * WHO CNS grade 4 neoplasm. | ||
* Up to 15% of all hemispheric pediatric gliomas. | * Up to 15% of all hemispheric pediatric gliomas. | ||
* Highly cellular tumor often with microvascular proliferation and necrosis. | * Highly cellular tumor often with microvascular proliferation and necrosis. | ||
* May resemble embryonal tumours (important DDx). | * May resemble embryonal tumours (important DDx).<ref name="pmid26482474">Korshunov A, Capper D, Reuss D, Schrimpf D, Ryzhova M, Hovestadt V | display-authors=etal (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=26482474 Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity.] ''Acta Neuropathol'' 131 (1):137-46. [http://dx.doi.org/10.1007/s00401-015-1493-1 DOI:10.1007/s00401-015-1493-1] PMID: [https://pubmed.gov/26482474 26482474]</ref> | ||
* IHC: MAP2+ve, TP53+ve, Olig2-ve, ATRX loss. | * IHC: MAP2+ve, TP53+ve, Olig2-ve, ATRX loss.<ref name="pmid34352809">Wang L, Shao L, Li H, Yao K, Duan Z, Zhi C | display-authors=etal (2022) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=34352809 Histone H3.3 G34-mutant Diffuse Gliomas in Adults.] ''Am J Surg Pathol'' 46 (2):249-257. [http://dx.doi.org/10.1097/PAS.0000000000001781 DOI:10.1097/PAS.0000000000001781] PMID: [https://pubmed.gov/34352809 34352809]</ref> | ||
* H3F3A G43R or G34V mutation present. | * H3F3A G43R or G34V mutation present. | ||
==References | =Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype= | ||
* CNS WHO grade 4 tumour ocurring in children and adolescents. | |||
* May occur anywhere in the brain. | |||
* Contrast enhancing tumour. | |||
* Frequent alterations include TP53, PDGFRA, EGFR or MYCN. | |||
* Absence of IDH1/2 and H3F3a mutation. | |||
* Three distinct subtypes in methylation profile. | |||
=Infant-type hemispheric glioma= | |||
* Large astrocytic tumor. | |||
* Median age: 2 months. | |||
* Supratentorial. | |||
* May be present before birth. | |||
* Well demarcated. | |||
* Typically with ALK, ROS1 or MET fusions. | |||
* Distinct methylation tumor profile. | |||
* Outcome in infants with ALK fusions usu. somewhat better than in older children. | |||
* IHC: GFAP +/-ve, Olig2+ve | |||
=See also= | |||
*[[Pediatric-type diffuse low-grade glioma]]. | |||
=References= | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology tumours]] | [[Category:Neuropathology tumours]] | ||