Difference between revisions of "Angiocentric glioma"

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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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}}
}}


'''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==
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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==

Latest revision as of 14:18, 20 November 2019

Angiocentric glioma
Diagnosis in short

Angiocentric glioma. H&E stain.
LM DDx astrocytoma, ependymoma.
IHC GFAP +/-ve, EMA +/-ve.
Gross enlargened gyri
Site brain - usu. grey matter
Clinical history epilepsy-associated
Prevalence very rare - no age prevalence
Prognosis good (WHO Grade I)


Angiocentric glioma, 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.[1]
  • Low-grade glioma - WHO Grade I by definition, but a single recurrence with anaplastic features has been described.[2]
  • 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.
  • Variably GFAP, EMA and S-100 positive
  • No IDH1/2 mutations. [3]
  • MIB-1 between 1-5%

DDx of angiocentric glioma (brief):

Molecular

  • Deletion-truncation breakpoints in MYB/MYBL on 6q23-[4]
  • MYB-QKI rearrangements [5]

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.[6]


Images

  • Angiocentric glioma - low mag. (WC/jensflorian)

  • Angiocentric glioma - intermed mag. (WC/jensflorian)

  • Angiocentric glioma - high mag. (WC/jensflorian)

  • Angiocentric glioma - GFAP immunostain (WC/jensflorian)

  • Angiocentric glioma - EMA immunostain (WC/jensflorian)

  • Angiocentric glioma - MAP2 immunostain (WC/jensflorian)

  • Angiocentric glioma - MIB-1 immunostain (WC/jensflorian)

See also

References

  1. ↑ Brat, DJ.; Scheithauer, BW.; Fuller, GN.; Tihan, T. (Jul 2007). "Newly codified glial neoplasms of the 2007 WHO Classification of Tumours of the Central Nervous System: angiocentric glioma, pilomyxoid astrocytoma and pituicytoma.". Brain Pathol 17 (3): 319-24. doi:10.1111/j.1750-3639.2007.00082.x. PMID 17598825.
  2. ↑ Wang, M.; Tihan, T.; Rojiani, AM.; Bodhireddy, SR.; Prayson, RA.; Iacuone, JJ.; Alles, AJ.; Donahue, DJ. et al. (Oct 2005). "Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma.". J Neuropathol Exp Neurol 64 (10): 875-81. PMID 16215459.
  3. ↑ Raghunathan, A.; Olar, A.; Vogel, H.; Parker, JR.; Coventry, SC.; Debski, R.; Albarracin, CT.; Aldape, KD. et al. (Aug 2012). "Isocitrate dehydrogenase 1 R132H mutation is not detected in angiocentric glioma.". Ann Diagn Pathol 16 (4): 255-9. doi:10.1016/j.anndiagpath.2011.11.003. PMID 22445362.
  4. ↑ Ramkissoon, LA.; Horowitz, PM.; Craig, JM.; Ramkissoon, SH.; Rich, BE.; Schumacher, SE.; McKenna, A.; Lawrence, MS. et al. (May 2013). "Genomic analysis of diffuse pediatric low-grade gliomas identifies recurrent oncogenic truncating rearrangements in the transcription factor MYBL1.". Proc Natl Acad Sci U S A 110 (20): 8188-93. doi:10.1073/pnas.1300252110. PMID 23633565.
  5. ↑ Bandopadhayay, P.; Ramkissoon, LA.; Jain, P.; Bergthold, G.; Wala, J.; Zeid, R.; Schumacher, SE.; Urbanski, L. et al. (Mar 2016). "MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism.". Nat Genet 48 (3): 273-82. doi:10.1038/ng.3500. PMID 26829751.
  6. ↑ Chiang, J.; Harreld, JH.; Tinkle, CL.; Moreira, DC.; Li, X.; Acharya, S.; Qaddoumi, I.; Ellison, DW. (Dec 2019). "A single-center study of the clinicopathologic correlates of gliomas with a MYB or MYBL1 alteration.". Acta Neuropathol 138 (6): 1091-1092. doi:10.1007/s00401-019-02081-1. PMID 31595312.
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