Angiocentric glioma

From Libre Pathology
Jump to: navigation, search
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]


Images


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.