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→Molecular: Morphological spectrum
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| Micro = | | Micro = | ||
| Subtypes = | | Subtypes = | ||
| LMDDx = [[astrocytoma]], [[ependymoma] | | LMDDx = [[astrocytoma]], [[ependymoma]]. | ||
| Stains = | | Stains = | ||
| IHC = GFAP +/-ve, EMA +/-ve. | | IHC = GFAP +/-ve, [[EMA]] +/-ve. | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
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}} | }} | ||
'''Angiocentric glioma | '''Angiocentric glioma''', is a WHO grade I [[glioma]]. It is super rare. | ||
==General== | ==General== | ||
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*Low cellularity and rich myxoid background- when compared to classical astrocytomas. | *Low cellularity and rich myxoid background- when compared to classical astrocytomas. | ||
**Mitotic activity may lead to eroneous diagnosis of [[anaplastic astrocytoma]]. | **Mitotic activity may lead to eroneous diagnosis of [[anaplastic astrocytoma]]. | ||
*Variably GFAP, EMA and S-100 positive | *Variably GFAP, [[EMA]] and S-100 positive | ||
* No IDH1/2 mutations. <ref>{{Cite journal | last1 = Raghunathan | first1 = A. | last2 = Olar | first2 = A. | last3 = Vogel | first3 = H. | last4 = Parker | first4 = JR. | last5 = Coventry | first5 = SC. | last6 = Debski | first6 = R. | last7 = Albarracin | first7 = CT. | last8 = Aldape | first8 = KD. | last9 = Cahill | first9 = DP. | title = Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. | journal = Ann Diagn Pathol | volume = 16 | issue = 4 | pages = 255-9 | month = Aug | year = 2012 | doi = 10.1016/j.anndiagpath.2011.11.003 | PMID = 22445362 }}</ref> | * No IDH1/2 mutations. <ref>{{Cite journal | last1 = Raghunathan | first1 = A. | last2 = Olar | first2 = A. | last3 = Vogel | first3 = H. | last4 = Parker | first4 = JR. | last5 = Coventry | first5 = SC. | last6 = Debski | first6 = R. | last7 = Albarracin | first7 = CT. | last8 = Aldape | first8 = KD. | last9 = Cahill | first9 = DP. | title = Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma. | journal = Ann Diagn Pathol | volume = 16 | issue = 4 | pages = 255-9 | month = Aug | year = 2012 | doi = 10.1016/j.anndiagpath.2011.11.003 | PMID = 22445362 }}</ref> | ||
*MIB-1 between 1-5% | *MIB-1 between 1-5% | ||
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*[[Ependymoma]]. | *[[Ependymoma]]. | ||
*[[Astrocytoma]]. | *[[Astrocytoma]]. | ||
* Isomorphic astrocytoma. | |||
==Molecular== | ==Molecular== | ||
* Deletion-truncation breakpoints in MYB/MYBL on 6q23-<ref>{{Cite journal | last1 = Ramkissoon | first1 = LA. | last2 = Horowitz | first2 = PM. | last3 = Craig | first3 = JM. | last4 = Ramkissoon | first4 = SH. | last5 = Rich | first5 = BE. | last6 = Schumacher | first6 = SE. | last7 = McKenna | first7 = A. | last8 = Lawrence | first8 = MS. | last9 = Bergthold | first9 = G. | title = Genomic analysis of diffuse pediatric low-grade gliomas identifies recurrent oncogenic truncating rearrangements in the transcription factor MYBL1. | journal = Proc Natl Acad Sci U S A | volume = 110 | issue = 20 | pages = 8188-93 | month = May | year = 2013 | doi = 10.1073/pnas.1300252110 | PMID = 23633565 }}</ref> | * Deletion-truncation breakpoints in MYB/MYBL on 6q23-<ref>{{Cite journal | last1 = Ramkissoon | first1 = LA. | last2 = Horowitz | first2 = PM. | last3 = Craig | first3 = JM. | last4 = Ramkissoon | first4 = SH. | last5 = Rich | first5 = BE. | last6 = Schumacher | first6 = SE. | last7 = McKenna | first7 = A. | last8 = Lawrence | first8 = MS. | last9 = Bergthold | first9 = G. | title = Genomic analysis of diffuse pediatric low-grade gliomas identifies recurrent oncogenic truncating rearrangements in the transcription factor MYBL1. | journal = Proc Natl Acad Sci U S A | volume = 110 | issue = 20 | pages = 8188-93 | month = May | year = 2013 | doi = 10.1073/pnas.1300252110 | PMID = 23633565 }}</ref> | ||
* MYB-QKI rearrangements <ref>{{Cite journal | last1 = Bandopadhayay | first1 = P. | last2 = Ramkissoon | first2 = LA. | last3 = Jain | first3 = P. | last4 = Bergthold | first4 = G. | last5 = Wala | first5 = J. | last6 = Zeid | first6 = R. | last7 = Schumacher | first7 = SE. | last8 = Urbanski | first8 = L. | last9 = O'Rourke | first9 = R. | title = MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism. | journal = Nat Genet | volume = 48 | issue = 3 | pages = 273-82 | month = Mar | year = 2016 | doi = 10.1038/ng.3500 | PMID = 26829751 }}</ref> | |||
Note: Gliomas with MYB/MYBL alteration may histologically resemble diffuse astrocytoma in children but these tumors cluster together with angiocentric glioma suggesting a single tumor entity.<ref>{{Cite journal | last1 = Chiang | first1 = J. | last2 = Harreld | first2 = JH. | last3 = Tinkle | first3 = CL. | last4 = Moreira | first4 = DC. | last5 = Li | first5 = X. | last6 = Acharya | first6 = S. | last7 = Qaddoumi | first7 = I. | last8 = Ellison | first8 = DW. | title = A single-center study of the clinicopathologic correlates of gliomas with a MYB or MYBL1 alteration. | journal = Acta Neuropathol | volume = 138 | issue = 6 | pages = 1091-1092 | month = Dec | year = 2019 | doi = 10.1007/s00401-019-02081-1 | PMID = 31595312 }}</ref> | |||
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Image:Neuropathology_case_V_02.jpg | Angiocentric glioma - intermed mag. (WC/jensflorian) | Image:Neuropathology_case_V_02.jpg | Angiocentric glioma - intermed mag. (WC/jensflorian) | ||
Image:Neuropathology_case_V_03.jpg | Angiocentric glioma - high mag. (WC/jensflorian) | Image:Neuropathology_case_V_03.jpg | Angiocentric glioma - high mag. (WC/jensflorian) | ||
Image:Neuropathology_case_V_04.jpg | Angiocentric glioma - EMA immunostain (WC/jensflorian) | Image:Neuropathology_case_V_04.jpg | Angiocentric glioma - GFAP immunostain (WC/jensflorian) | ||
Image:Neuropathology_case_V_05.jpg | Angiocentric glioma - [[EMA]] immunostain (WC/jensflorian) | |||
Image:Neuropathology_case_V_06.jpg | Angiocentric glioma - MAP2 immunostain (WC/jensflorian) | |||
Image:Neuropathology_case_V_07.jpg | Angiocentric glioma - MIB-1 immunostain (WC/jensflorian) | |||
</gallery> | </gallery> | ||
==See also== | ==See also== | ||