Angiocentric glioma - Revision history

Jensflorian: /* Molecular */ Morphological spectrum

2019-11-20T14:18:47Z

Molecular: Morphological spectrum

← Older revision		Revision as of 14:18, 20 November 2019
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	* 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>		* 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>

Jensflorian: /* Microscopic */ DDx

2019-03-25T13:54:54Z

Microscopic: DDx

← Older revision		Revision as of 13:54, 25 March 2019
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	*[[Ependymoma]].		*[[Ependymoma]].
	*[[Astrocytoma]].		*[[Astrocytoma]].
			* Isomorphic astrocytoma.

	==Molecular==		==Molecular==

Jensflorian: /* Molecular */

2019-03-25T13:52:11Z

Molecular

← Older revision		Revision as of 13:52, 25 March 2019
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	==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>


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	Image:Neuropathology_case_V_07.jpg \| Angiocentric glioma - MIB-1 immunostain (WC/jensflorian)		Image:Neuropathology_case_V_07.jpg \| Angiocentric glioma - MIB-1 immunostain (WC/jensflorian)
	</gallery>		</gallery>



	==See also==		==See also==

Jensflorian: wikify

2015-09-23T10:52:56Z

wikify

← Older revision		Revision as of 10:52, 23 September 2015
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	\| 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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	*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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	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 - GFAP 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_05.jpg \| Angiocentric glioma - [[EMA]] immunostain (WC/jensflorian)
	Image:Neuropathology_case_V_06.jpg \| Angiocentric glioma - MAP2 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)		Image:Neuropathology_case_V_07.jpg \| Angiocentric glioma - MIB-1 immunostain (WC/jensflorian)

Jensflorian: more images added

2015-09-23T09:49:22Z

more images added

← Older revision		Revision as of 09:49, 23 September 2015
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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>

Jensflorian: fix 2

2015-09-23T09:46:38Z

fix 2

← Older revision		Revision as of 09:46, 23 September 2015
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	}}		}}

	'''Angiocentric glioma~~''', abbreviated '''PA~~''', is a WHO grade I [[glioma]]. It is super rare.		'''Angiocentric glioma''', is a WHO grade I [[glioma]]. It is super rare.

	==General==		==General==

Jensflorian: fix

2015-09-23T09:45:58Z

fix

← Older revision		Revision as of 09:45, 23 September 2015
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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.

Jensflorian: created

2015-09-23T09:44:50Z

created

New page

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Neuropathology_case_V_03.jpg
| Width =
| Caption = Angiocentric glioma. [[H&E stain]].
| Synonyms =
| Micro =
| Subtypes =
| LMDDx = [[astrocytoma]], [[ependymoma]
| Stains =
| IHC = GFAP +/-ve, EMA +/-ve.
| EM =
| Molecular =
| IF =
| Gross = enlargened gyri
| Grossing =
| Site = brain - usu. grey matter
| Assdx =
| Syndromes =
| Clinicalhx = epilepsy-associated
| Signs =
| Symptoms =
| Prevalence = very rare - no age prevalence
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = good (WHO Grade I)
| Other =
| ClinDDx =
| Tx =
}}

'''Angiocentric glioma''', abbreviated '''PA''', is a WHO grade I [[glioma]]. It is super rare.

==General==
* previously called monomorphic angiocentric glioma or angiocentric neuroepithelial tumour.
* Own entity introduced in the WHO 2007 classification.<ref>{{Cite journal | last1 = Brat | first1 = DJ. | last2 = Scheithauer | first2 = BW. | last3 = Fuller | first3 = GN. | last4 = Tihan | first4 = T. | title = Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma. | journal = Brain Pathol | volume = 17 | issue = 3 | pages = 319-24 | month = Jul | year = 2007 | doi = 10.1111/j.1750-3639.2007.00082.x | PMID = 17598825 }}</ref>
*Low-grade [[glioma]] - ''WHO Grade I'' by definition, but a single recurrence with anaplastic features has been described.<ref>
{{Cite journal | last1 = Wang | first1 = M. | last2 = Tihan | first2 = T. | last3 = Rojiani | first3 = AM. | last4 = Bodhireddy | first4 = SR. | last5 = Prayson | first5 = RA. | last6 = Iacuone | first6 = JJ. | last7 = Alles | first7 = AJ. | last8 = Donahue | first8 = DJ. | last9 = Hessler | first9 = RB. | title = Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. | journal = J Neuropathol Exp Neurol | volume = 64 | issue = 10 | pages = 875-81 | month = Oct | year = 2005 | doi = | PMID = 16215459 }}</ref>
*Classically a non-enhancing, superficial cerebrocortical lesion.
*Associated with epilepsy.
*No association with any tumour syndromes.

==Gross==
*Usually well-circumscribed.
*Enlargement of cortex possible.

==Microscopic==
Features:
*Monommorphic, bipolar, spindled cells around blood vessels.
**mimicking ependymal pseudorosettes (DD: ependymoma).
*Solid growth with palisaded arrays possible.
*Low cellularity and rich myxoid background- when compared to classical astrocytomas.
**Mitotic activity may lead to eroneous diagnosis of [[anaplastic astrocytoma]].
*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>
*MIB-1 between 1-5%

DDx of angiocentric glioma (brief):
*[[Pilomyxoid astrocytoma]]
*[[Ependymoma]].
*[[Astrocytoma]].

==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>

===Images===
<gallery>
Image:Neuropathology_case_V_01.jpg | Angiocentric glioma - low 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_04.jpg | Angiocentric glioma - EMA immunostain (WC/jensflorian)
</gallery>

==See also==
*[[Neuropathology tumours]].
*[[Astrocytoma]].

==References==
{{Reflist|2}}

[[Category:Diagnosis]]
[[Category:Neuropathology tumours]]