Difference between revisions of "Pilocytic astrocytoma"

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small update (molecular, sections)
(small update (molecular, sections))
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==General==
==General==
*Low-grade [[astrocytoma]] - ''WHO Grade I'' by definition.
*Low-grade [[astrocytoma]] - ''WHO Grade I'' by definition, but rare anaplastic forms have been described.
*Classically in the cerebellum in children; most common glioma in children.<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref>
*Classically in the cerebellum in children; most common glioma in children.<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref>
*The ''optic glioma'' associated with neurofibromatosis 1.
*The ''optic glioma'' is associated with neurofibromatosis 1.
*Usually enhances after CM application


==Gross==
==Gross==
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*Chronic reactive gliosis.
*Chronic reactive gliosis.
*Subependymoma.
*Subependymoma.
*Ganglioma.
*Ganglioglioma.
*Alexander's disease (rare leukodystrophy).
*Alexander's disease (rare leukodystrophy).


DDx of pilocystic astrocytoma (brief):
DDx of pilocystic astrocytoma (brief):
*Piloid gliosis.
*Piloid gliosis (esp. in sellar lesions).
*[[Oligodendroglioma]].
*[[Oligodendroglioma]].
*[[Glioblastoma]] (uncommon - but important).
*[[Glioblastoma]] (uncommon - but important).
*Tanycytic [[Ependymoma]]
*Pilocytic tumor components may be found in [[Ganglioglioma]], [[DNET]], [[RGNT]]


===Images===  
===Images===  
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<gallery>
<gallery>
Image:Rosenthal_HE_40x.jpg | Rosenthal fibres. (WC)
Image:Rosenthal_HE_40x.jpg | Rosenthal fibres. (WC)
Image:Pilocytic astrocytoma cell pleomorphism.jpg | Occasional plomorphism (WC)
Image:Pilocytic astrocytoma endothelial proliferations.jpg | Microvascular proliferation (WC)
</gallery>
</gallery>
www:
www:
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*CD68: may have a significant macrophage component.
*CD68: may have a significant macrophage component.
*KI-67: may be "high" (~20% ???).
*KI-67: may be "high" (~20% ???).
*Olig 2: Usually strongly present <ref>Otero J, J Neurooncol 2011, doi: 10.1007/s11060-010-0509-x</ref>.
==Molecular==
* alteration usually associated with the MAPK pathway
* KIAA1549-BRAF fusion transcripts most common in sporadic PA
* rarely BRAF mutations, SRGAP3-RAF1 or FAM131B-BRAF fusions


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