Difference between revisions of "Diffuse astrocytoma, MYB- or MYBL-altered"
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{{ Infobox diagnosis | {{ Infobox diagnosis | ||
| Name = {{PAGENAME}} | | Name = {{PAGENAME}} | ||
| Image = | | Image = Commons 20220404 000.jpg | ||
| Width = | | Width = | ||
| Caption = Diffuse astrocytoma, MYB- or MYBL-altered [[H&E stain]]. | | Caption = Diffuse astrocytoma, MYB- or MYBL-altered [[H&E stain]]. | ||
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* Entity introduced in 2021. <ref>{{cite journal |vauthors=Bale TA, Rosenblum MK |title=The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low-grade gliomas and glioneuronal tumors |journal=Brain Pathol |volume= |issue= |pages=e13060 |date=February 2022 |pmid=35218102 |doi=10.1111/bpa.13060 |url=}}</ref> | * Entity introduced in 2021. <ref>{{cite journal |vauthors=Bale TA, Rosenblum MK |title=The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low-grade gliomas and glioneuronal tumors |journal=Brain Pathol |volume= |issue= |pages=e13060 |date=February 2022 |pmid=35218102 |doi=10.1111/bpa.13060 |url=}}</ref> | ||
* Rare epilepsy-associated tumor. | * Rare epilepsy-associated tumor. | ||
* Median time to surgery often exceeds more than 10 years. | |||
* No sex predilection. | * No sex predilection. | ||
| Line 64: | Line 65: | ||
===Images=== | ===Images=== | ||
<gallery> | <gallery> | ||
File:Commons 20220404 000.jpg|H&E | |||
</gallery> | </gallery> | ||
==Stains== | ==Stains== | ||
Features: | Features: | ||
*GFAP +ve. | *GFAP +ve. | ||
| Line 86: | Line 84: | ||
==Prognosis== | ==Prognosis== | ||
*Excellent ( | * Excellent (most cases are stable after surgery). | ||
==See also== | ==See also== | ||
Latest revision as of 07:50, 4 April 2022
| Diffuse astrocytoma, MYB- or MYBL-altered | |
|---|---|
| Diagnosis in short | |
 Diffuse astrocytoma, MYB- or MYBL-altered H&E stain. | |
| LM DDx | Angiocentric glioma |
| IHC | GFAP +ve |
| Gross | soft, unencapsulated |
| Site | brain - usu. temporal |
|
| |
| Prevalence | common - esp. in children |
| Prognosis | good (WHO Grade I) |
Diffuse astrocytoma, MYB- or MYBL-altered is a low-grade, often pediatric-onset astrocytoma. It is very rare.
General
- CNS WHO grade 1.
- Entity introduced in 2021. [1]
- Rare epilepsy-associated tumor.
- Median time to surgery often exceeds more than 10 years.
- No sex predilection.
Imaging
- T1 hypointense.
- Non-enhancing.
- Occasionally large cysts.
Gross
- Soft, unencapsulated.
- Grey to white.
Microscopic
Features:
- Diffusely growing.
- Minimal incereased cell density.
- Monomorphic, bland glial cells.
- Entrapped neurons.
- Usu. no mitotic activity
Notes:
- Focally angiocentric pattern may be present.
DDx
- Normal white matter
- Angiocentric glioma
Images
H&E
Stains
Features:
- GFAP +ve.
- Olig2 -ve.
- MAP2 -ve.
- CD34 -ve.
- ATRX retained.
Molecular
- Structural variant of MYB or MYBL1 (most common fusion partners: MAML2, MMP16 , PCDGHA1).[2]
- IDH1/2 wildtype.
- H3F3A wildtype.
Prognosis
- Excellent (most cases are stable after surgery).
See also
References
- ↑ "The 2021 WHO Classification of Tumors of the Central Nervous System: An update on pediatric low-grade gliomas and glioneuronal tumors". Brain Pathol: e13060. February 2022. doi:10.1111/bpa.13060. PMID 35218102.
- ↑ "Genetic alterations in uncommon low-grade neuroepithelial tumors: BRAF, FGFR1, and MYB mutations occur at high frequency and align with morphology". Acta Neuropathol 131 (6): 833–45. June 2016. doi:10.1007/s00401-016-1539-z. PMC 4866893. PMID 26810070. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4866893/.