High-grade astrocytoma with piloid features - Revision history

Jensflorian: HGAP

2022-10-17T13:54:06Z

HGAP

← Older revision		Revision as of 13:54, 17 October 2022
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	}}		}}

	'''High-grade astrocytoma with piloid features''' is a rare [[Glioma\|glial]] tumor which often requires methylation analyis to secure diagnosis. There is currently no definitive grading, but clinical behaviour suggests WHO CNS grade 3.		'''High-grade astrocytoma with piloid features''' (HGAP) is a rare [[Glioma\|glial]] tumor which often requires methylation analyis to secure diagnosis. There is currently no definitive grading, but clinical behaviour suggests WHO CNS grade 3.


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	==Images==		==Images==
	<gallery>		<gallery>
	Image:Pilocytic astrocytoma with anaplastic features.jpg \| ~~APA~~, [[FFPE]] specimen, HE (WC).		Image:Pilocytic astrocytoma with anaplastic features.jpg \| HGAP, [[FFPE]] specimen, HE (WC).
	Image:Pilocytic astrocytoma with anaplastic features mimicking focally a GBM.jpg \| Vacular proliferations and necrosis in ~~APA~~ mimicking [[GBM]] (WC).		Image:Pilocytic astrocytoma with anaplastic features mimicking focally a GBM.jpg \| Vacular proliferations and necrosis in HGAP mimicking [[GBM]] (WC).
	</gallery>		</gallery>

Jensflorian: Update infobox

2022-10-17T13:47:41Z

Update infobox

← Older revision		Revision as of 13:47, 17 October 2022
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			{{ Infobox diagnosis
			\| Name = {{PAGENAME}}
			\| Image = Pilocytic astrocytoma with anaplastic features.jpg
			\| Width =
			\| Caption = High-grade astrocytoma with piloid features. [[H&E stain]].
			\| Synonyms =
			\| Micro =
			\| Subtypes =
			\| LMDDx = [[astrocytoma]], [[PXA]], [[glioblastoma]]
			\| Stains = PAS-D +ve (eosinophilic granular bodies)
			\| IHC = GFAP +ve
			\| EM =
			\| Molecular =
			\| IF =
			\| Gross = usually cerebellar
			\| Grossing =
			\| Site = brain - usu. [[cerebellum]]
			\| Assdx =
			\| Syndromes =
			\| Clinicalhx =
			\| Signs =
			\| Symptoms =
			\| Prevalence = very rare - esp. in children
			\| Bloodwork =
			\| Rads =
			\| Endoscopy =
			\| Prognosis = poor (analog to WHO Grade III)
			\| Other =
			\| ClinDDx =
			\| Tx =
			}}

	'''High-grade astrocytoma with piloid features''' is a rare [[Glioma\|glial]] tumor which often requires methylation analyis to secure diagnosis. There is currently no definitive grading, but clinical behaviour suggests WHO CNS grade 3.		'''High-grade astrocytoma with piloid features''' is a rare [[Glioma\|glial]] tumor which often requires methylation analyis to secure diagnosis. There is currently no definitive grading, but clinical behaviour suggests WHO CNS grade 3.

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	*Imaging may be similiar to [[Glioblastoma]].		*Imaging may be similiar to [[Glioblastoma]].
	*5-year OS: 50%.		*5-year OS: 50%.
			*De novo cases in the setting of neurofibromatosis 1 reported.
			*Tumors may have history of previous radiation.

	=Histology=		=Histology=
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	*Perivascular lymphocytic cuffing.		*Perivascular lymphocytic cuffing.
	*Necrosis may be present.		*Necrosis may be present.

			==Images==
			<gallery>
			Image:Pilocytic astrocytoma with anaplastic features.jpg \| APA, [[FFPE]] specimen, HE (WC).
			Image:Pilocytic astrocytoma with anaplastic features mimicking focally a GBM.jpg \| Vacular proliferations and necrosis in APA mimicking [[GBM]] (WC).
			</gallery>

	=IHC=		=IHC=
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	*TERT promotor mutations are rare.		*TERT promotor mutations are rare.
	*Absence of IDH1/2 hotspot mutation.		*Absence of IDH1/2 hotspot mutation.

			=Prognosis=
			*Poor, but better than conventional glioblastoma.<ref>{{Cite journal \| last1 = Rodriguez \| first1 = FJ. \| last2 = Scheithauer \| first2 = BW. \| last3 = Burger \| first3 = PC. \| last4 = Jenkins \| first4 = S. \| last5 = Giannini \| first5 = C. \| title = Anaplasia in pilocytic astrocytoma predicts aggressive behavior. \| journal = Am J Surg Pathol \| volume = 34 \| issue = 2 \| pages = 147-60 \| month = Feb \| year = 2010 \| doi = 10.1097/PAS.0b013e3181c75238 \| PMID = 20061938 }}</ref>
			*Prognostic unfavourable paramters include: Necrosis, Mitoses and previous radiation.


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

Jensflorian: Category

2022-04-13T11:44:48Z

Jensflorian: Create

2022-04-13T11:43:49Z

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'''High-grade astrocytoma with piloid features''' is a rare [[Glioma|glial]] tumor which often requires methylation analyis to secure diagnosis. There is currently no definitive grading, but clinical behaviour suggests WHO CNS grade 3.

=Clinical=
*Rare (1-3% of all brain tumor in adults).
*Usu. posterior fossa (75%).
*Supratentorial or spinal locations possible.
*Imaging may be similiar to [[Glioblastoma]].
*5-year OS: 50%.

=Histology=
*Often so variable, so molecular testing is essential to secure diagnosis.
*Astrocytic nature of tumor cells.
*Frequent mitoses.
*Elongated glial tumor cell processes ("piloid").
*Rosenthal fibers or eosinophilic granular bodies.
*Perivascular lymphocytic cuffing.
*Necrosis may be present.

=IHC=
*[[GFAP]]+ve.
*ATRX: nuclear loss in approx. 40%.

=Molecular=
*DNA Methylation profile of high-grade astrocytoma with piloid features is essential for diagnosis.
*MAPK genes often altered (NF1, BRAF fusion, FGFR1, KRAS).
*CDKN2A/B homozygous deletion.
*CDK4 amplification.
*TERT promotor mutations are rare.
*Absence of IDH1/2 hotspot mutation.

=DDx:=
*[[Glioblastoma]]
*[[Pleomorphic xanthoastrocytoma]]

← Older revision		Revision as of 11:44, 13 April 2022
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	*[[Glioblastoma]]		*[[Glioblastoma]]
	*[[Pleomorphic xanthoastrocytoma]]		*[[Pleomorphic xanthoastrocytoma]]

			=See also=
			*[[Astrocytoma]].
			*[[Neuropathology_tumours#Infiltrative_astrocytomas]]

			{{Reflist\|2}}

			[[Category:Diagnosis]]