Diffuse astrocytoma, MYB- or MYBL-altered

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Diffuse astrocytoma, MYB- or MYBL-altered
Diagnosis in short
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 pediatric astrocytoma. It is very rare.

General

Imaging

Gross

Microscopic

Features:[1]

  • Classically biphasic (though either may be absent):
    1. Fibrillar.
    2. Microcystic/loose.
  • Hair-like fibres ~ 1 micrometer; pilo- = hair.[2]
    • Best seen on smear or with GFAP IHC.
  • Rosenthal fibres - key feature.
    • May be rare. Not pathognomonic (see below).
  • Eosinophilic granular bodies.
  • Low cellularity - when compared to medulloblastoma and ependymoma.

Notes:

  • +/-Microvascular proliferation.
  • +/-Focal necrosis.
    • Necrosis with pseudopalisading more likely glioblastoma.
  • +/-Mitoses - not significant in the context of the Dx.

DDx

  • Normal white matter
  • Angiocentric glioma


Images

Smears


Stains

IHC

Features:

  • GFAP +ve (fibres).


Molecular

Prognosis

  • Excellent (

See also

References

  1. Perry, Arie; Brat, Daniel J. (2010). Practical Surgical Neuropathology: A Diagnostic Approach: A Volume in the Pattern Recognition series (1st ed.). Churchill Livingstone. pp. 82-4. ISBN 978-0443069826.
  2. URL: http://dictionary.reference.com/browse/pilo-. Accessed on: 24 November 2010.