Ewing sarcoma - Revision history

Michael at 12:37, 15 October 2015

2015-10-15T12:37:30Z

← Older revision		Revision as of 12:37, 15 October 2015
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	\| LMDDx = [[Ewing-like tumours]] (e.g. [[CIC-DUX sarcoma]]), [[small round cell tumours]] ([[lymphoma]], [[small cell osteosarcoma]], others)		\| LMDDx = [[Ewing-like tumours]] (e.g. [[CIC-DUX sarcoma]]), [[small round cell tumours]] ([[lymphoma]], [[small cell osteosarcoma]], others)
	\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve		\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve
	\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve		\| IHC = [[CD99]] +ve, FLI-1 +ve, CD45 -ve
	\| EM =		\| EM =
	\| Molecular = EWS [[Chromosomal translocations\|translocation]], EWS/FLI-1 fusion gene in 90% of cases		\| Molecular = EWS [[Chromosomal translocations\|translocation]], EWS/FLI-1 fusion gene in 90% of cases
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	Notes:<ref>PST. 22 February 2010.</ref>		Notes:<ref>PST. 22 February 2010.</ref>
	*CD99 +ve		*[[CD99]] +ve
	**Plasma membrane staining tumours:		**Plasma membrane staining tumours:
	***[[Lymphoblastic lymphoma]]/leukemia.		***[[Lymphoblastic lymphoma]]/leukemia.

Michael: +DDx, Ewing-like link

2015-04-22T19:03:46Z

+DDx, Ewing-like link

← Older revision		Revision as of 19:03, 22 April 2015
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	\| Micro = [[small round cell tumour]], clear cytoplasm		\| Micro = [[small round cell tumour]], clear cytoplasm
	\| Subtypes =		\| Subtypes =
	\| LMDDx = [[small round cell tumours]] ([[lymphoma]], [[small cell osteosarcoma]], others)		\| LMDDx = [[Ewing-like tumours]] (e.g. [[CIC-DUX sarcoma]]), [[small round cell tumours]] ([[lymphoma]], [[small cell osteosarcoma]], others)
	\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve		\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve
	\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve		\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve
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	*It is a [[small round cell tumour]].		*It is a [[small round cell tumour]].
	*[[Rhabdomyosarcoma]] occasionally has cytoplasmic clearing (due to glycogen).<ref name=PST14feb11>PST. 14 February 2011.</ref>		*[[Rhabdomyosarcoma]] occasionally has cytoplasmic clearing (due to glycogen).<ref name=PST14feb11>PST. 14 February 2011.</ref>

			DDx:
			*[[Ewing-like tumours]] (e.g. [[CIC-DUX sarcoma]]).
			*Large cell [[lymphoma]].
			*Other [[small round cell tumours]] (e.g. small cell [[osteosarcoma]]).

	====Images====		====Images====

Michael: touch

2014-12-18T03:59:23Z

touch

← Older revision		Revision as of 03:59, 18 December 2014
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	{{ Infobox diagnosis		{{ Infobox diagnosis
	\| Name = {{PAGENAME}}		\| Name = {{PAGENAME}}
	\| Image = Ewing sarcoma - PAS - high mag.jpg		\| Image = Ewing sarcoma - PAS - high mag.jpg
	\| Width =		\| Width =
	\| Caption = Ewing sarcoma. [[PAS stain]].		\| Caption = Ewing sarcoma. [[PAS stain]].

Michael: fix infobox

2013-08-25T12:04:27Z

fix infobox

← Older revision		Revision as of 12:04, 25 August 2013
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	\| Image = Ewing sarcoma - PAS - high mag.jpg		\| Image = Ewing sarcoma - PAS - high mag.jpg
	\| Width =		\| Width =
	\| Caption = Ewing sarcoma. [[~~H&E~~ stain]].		\| Caption = Ewing sarcoma. [[PAS stain]].
	\| Micro = [[small round cell tumour]], clear cytoplasm		\| Micro = [[small round cell tumour]], clear cytoplasm
	\| Subtypes =		\| Subtypes =

Michael at 12:01, 25 August 2013

2013-08-25T12:01:00Z

← Older revision		Revision as of 12:01, 25 August 2013
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	\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve		\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve
	\| EM =		\| EM =
	\| Molecular = EWS [[~~molecular translocation~~\|translocation]], EWS/FLI-1 fusion gene in 90% of cases		\| Molecular = EWS [[Chromosomal translocations\|translocation]], EWS/FLI-1 fusion gene in 90% of cases
	\| IF =		\| IF =
	\| Gross =		\| Gross =

Michael: more stuff

2013-08-25T11:59:46Z

more stuff

← Older revision		Revision as of 11:59, 25 August 2013
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	{{ Infobox diagnosis		{{ Infobox diagnosis
	\| Name = {{PAGENAME}}		\| Name = {{PAGENAME}}
	\| Image =		\| Image = Ewing sarcoma - PAS - high mag.jpg
	\| Width =		\| Width =
	\| Caption =		\| Caption = Ewing sarcoma. [[H&E stain]].
	\| Micro =		\| Micro = [[small round cell tumour]], clear cytoplasm
	\| Subtypes =		\| Subtypes =
	\| LMDDx = [[small round cell tumours]]		\| LMDDx = [[small round cell tumours]] ([[lymphoma]], [[small cell osteosarcoma]], others)
	\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve		\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve
	\| IHC =		\| IHC = CD99 +ve, FLI-1 +ve, CD45 -ve
	\| EM =		\| EM =
	\| Molecular =		\| Molecular = EWS [[molecular translocation\|translocation]], EWS/FLI-1 fusion gene in 90% of cases
	\| IF =		\| IF =
	\| Gross =		\| Gross =
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	\| Assdx =		\| Assdx =
	\| Syndromes =		\| Syndromes =
	\| Clinicalhx =		\| Clinicalhx = usually <20 years old
	\| Signs =		\| Signs =
	\| Symptoms =		\| Symptoms =
	\| Prevalence =		\| Prevalence = uncommon
	\| Bloodwork =		\| Bloodwork =
	\| Rads =		\| Rads =
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	*Usually younger than 20 years.		*Usually younger than 20 years.
	*Second most common malignant bone tumour in children.		*Second most common malignant bone tumour in children.
	**Most common malignant bone tumour = osteosarcoma (AKA osteogenic sarcoma).		**Most common malignant bone tumour = [[osteosarcoma]] ([[AKA]] ''osteogenic sarcoma'').

	Poor prognostic factors:<ref>PST. 14 February 2011.</ref>		Poor prognostic factors:<ref>PST. 14 February 2011.</ref>
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	*CD45 -ve.		*CD45 -ve.
	**Done to r/o [[lymphoma]].		**Done to r/o [[lymphoma]].
	*+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), ~~S100~~).		*+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S-100).
	*+/-Cytokeratins.		*+/-Cytokeratins.
	*Caveolin-1 +ve in ~ 85% of EWS.<ref>PST. 14 February 2011.</ref>		*Caveolin-1 +ve in ~ 85% of EWS.<ref>PST. 14 February 2011.</ref>

Michael: +infobox

2013-08-25T11:50:04Z

+infobox

← Older revision		Revision as of 11:50, 25 August 2013
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			{{ Infobox diagnosis
			\| Name = {{PAGENAME}}
			\| Image =
			\| Width =
			\| Caption =
			\| Micro =
			\| Subtypes =
			\| LMDDx = [[small round cell tumours]]
			\| Stains = [[PAS stain]] +ve, [[PASD stain]] -ve
			\| IHC =
			\| EM =
			\| Molecular =
			\| IF =
			\| Gross =
			\| Grossing =
			\| Site = [[bone]], rarely other sites
			\| Assdx =
			\| Syndromes =
			\| Clinicalhx =
			\| Signs =
			\| Symptoms =
			\| Prevalence =
			\| Bloodwork =
			\| Rads =
			\| Endoscopy =
			\| Prognosis =
			\| Other =
			\| ClinDDx =
			}}
	'''Ewing sarcoma''', also known as '''EWS/pPNET''', is an uncommon bone tumour.		'''Ewing sarcoma''', also known as '''EWS/pPNET''', is an uncommon bone tumour.

Michael: wikify

2013-08-25T11:45:44Z

wikify

← Older revision		Revision as of 11:45, 25 August 2013
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	Confusingly, it is known as EWS/PNET. ''EWS'' is ''Ewing sarcoma''. ''pPNET'' is ''peripheral primitive neuroectodermal tumour''. ''EWS'' and ''pPNET'' were once thought to be different tumours.		Confusingly, it is known as EWS/PNET. ''EWS'' is ''Ewing sarcoma''. ''pPNET'' is ''peripheral primitive neuroectodermal tumour''. ''EWS'' and ''pPNET'' were once thought to be different tumours.

	''Peripheral primitive neuroectodermal tumour'' should not be confused with ''[[primitive neuroectodermal tumour]]'', commonly abbreviated ''PNET'', a (supertentorial) brain tumour with similarities to [[medulloblastoma]].		''Peripheral primitive neuroectodermal tumour'' should not be confused with ''[[primitive neuroectodermal tumour]]'', commonly abbreviated ''PNET'', a (supertentorial) [[brain tumour]] with similarities to [[medulloblastoma]].

	==General==		==General==

Michael: split out

2013-08-25T11:44:42Z

split out

← Older revision		Revision as of 11:44, 25 August 2013
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	~~#redirect~~ [[Chondro-osseous tumours~~#Ewing sarcoma~~]]		'''Ewing sarcoma''', also known as '''EWS/pPNET''', is an uncommon bone tumour.

			Confusingly, it is known as EWS/PNET. ''EWS'' is ''Ewing sarcoma''. ''pPNET'' is ''peripheral primitive neuroectodermal tumour''. ''EWS'' and ''pPNET'' were once thought to be different tumours.

			''Peripheral primitive neuroectodermal tumour'' should not be confused with ''[[primitive neuroectodermal tumour]]'', commonly abbreviated ''PNET'', a (supertentorial) brain tumour with similarities to [[medulloblastoma]].

			==General==
			Clinical:
			*Painful.
			*Usually younger than 20 years.
			*Second most common malignant bone tumour in children.
			**Most common malignant bone tumour = osteosarcoma (AKA osteogenic sarcoma).

			Poor prognostic factors:<ref>PST. 14 February 2011.</ref>
			*Age (18 years-old+).
			*Pelvis (extremity = good).
			*>8 cm.
			*[[Metastases]].
			*EWS-FL1 fusion type 2.
			*>90% [[necrosis]].

			Etiology:
			*Unknown origin; hypothesis: Ewing sarcoma arises from mesenchymal stem cell.<ref name=pmid20953407>{{cite journal \|author=Lin PP, Wang Y, Lozano G \|title=Mesenchymal Stem Cells and the Origin of Ewing's Sarcoma \|journal=Sarcoma \|volume=2011 \|issue= \|pages= \|year=2011 \|pmid=20953407 \|pmc=2952797 \|doi=10.1155/2011/276463 \|url=}}</ref>

			===Radiology===
			Features:<ref name=Ref_WMSP650>{{Ref WMSP\|650}}</ref>
			*Long bones, diaphyses.
			*Destructive.
			*"Onion-skin" periosteal reaction.

			==Microscopic==
			Features:<ref>PST. 22 February 2010.</ref>
			*Scant clear cytoplasm (contain glycogen -- PAS +ve, PAS-D -ve) - '''key feature'''.
			*Round small nucleus.
			**Usu. lack nucleoli.
			**Usu. minimal-moderate size variation.
			*Mitoses (common).

			Notes:
			*It is a [[small round cell tumour]].
			*[[Rhabdomyosarcoma]] occasionally has cytoplasmic clearing (due to glycogen).<ref name=PST14feb11>PST. 14 February 2011.</ref>

			====Images====
			<gallery>
			Image:Ewing sarcoma - intermed mag.jpg \| ES - intermed. mag.
			Image:Ewing sarcoma - high mag.jpg \| ES - high mag.
			Image:Ewing sarcoma - very high mag.jpg \| ES - very high mag.
			</gallery>
			<gallery>
			Image:Ewing sarcoma - PAS - intermed mag.jpg \| ES - PAS - intermed. mag.
			Image:Ewing sarcoma - PAS - high mag.jpg \| ES - PAS - high mag.
			Image:Ewing sarcoma - PAS - very high mag.jpg \| ES - PAS - very high mag.
			</gallery>
			www:
			*[http://path.upmc.edu/cases/case103.html Ewing sarcoma - case 1 - several images (upmc.edu)].
			*[http://path.upmc.edu/cases/case420.html Ewing sarcoma - case 2 - several images (upmc.edu)].

			==[[IHC]]==
			Features:<ref name=Ref_WMSP651>{{Ref WMSP\|651}}</ref>
			*CD99 +ve -- 1. diffuse, 2. plasma membrane staining; both required -- most specific.
			*FLI-1 +ve.<ref name=pmid15001993>{{cite journal \|author=Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos AP \|title=Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody \|journal=Mod. Pathol. \|volume=17 \|issue=5 \|pages=547–52 \|year=2004 \|month=May \|pmid=15001993 \|doi=10.1038/modpathol.3800065 \|url=http://www.nature.com/modpathol/journal/v17/n5/full/3800065a.html}}</ref>
			*CD45 -ve.
			**Done to r/o [[lymphoma]].
			*+/-Neural markers (NSE, synaptophysin, CD57 (??? CD56 ???), S100).
			*+/-Cytokeratins.
			*Caveolin-1 +ve in ~ 85% of EWS.<ref>PST. 14 February 2011.</ref>
			*WT-1 -ve.<ref name=Ref_WMSP286>{{Ref WMSP\|286}}</ref>

			Notes:<ref>PST. 22 February 2010.</ref>
			*CD99 +ve
			**Plasma membrane staining tumours:
			***[[Lymphoblastic lymphoma]]/leukemia.
			***[[Angiomatoid fibrous histiocytoma]].
			***[[Desmoplastic small round cell tumour]].
			**Weak/cytoplasmic staining:
			***[[Synovial sarcoma]].
			***[[Rhabdomyosarcoma]].
			***[[Rhabdoid tumour]].
			*FLI-1 +ve:<ref name=pmid15001993/>
			**[[Vascular neoplasms]].
			**-/+[[Merkel cell carcinoma]].
			**-/+[[Melanoma]].

			==Molecular diagnostics==
			Common features:
			*''EWS/FLI-1 fusion gene'' formation due to [[translocation]]: ''t(11;22)(q24;q12)''.<ref>URL: [http://atlasgeneticsoncology.org/Tumors/Ewing5010.html http://atlasgeneticsoncology.org/Tumors/Ewing5010.html]. Accessed on: 23 February 2010.</ref><ref name=pmid3163261>{{cite journal \|author=Turc-Carel C, Aurias A, Mugneret F, ''et al.'' \|title=Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12) \|journal=Cancer Genet. Cytogenet. \|volume=32 \|issue=2 \|pages=229–38 \|year=1988 \|month=June \|pmid=3163261 \|doi= \|url=}}</ref>
			**Often detected by RT-PCR (with EWS 5' and FLI-1 3' primers).
			**Type 1 = EWS exon 7 + FLI-1 exon 6; good prognosis.
			**Type 2 = others; poor prognosis.

			Notes:
			*The ''t(11;22)(q24;q12)'' is seen in ~90% of EWS/PNET... but also in:
			**[[Olfactory neuroblastoma]].
			**Small cell osteogenic sarcoma (small cell variant of [[osteosarcoma]]).
			**Polyphenotypic tumours.
			**[[Rhabdomyosarcoma]].
			**[[Neuroblastoma]] (possibly).
			*Several other EWS translocations exist.<ref>URL: [http://www.cancerindex.org/geneweb/EWSR1.htm http://www.cancerindex.org/geneweb/EWSR1.htm]. Accessed on: 20 November 2011.</ref>
			**ERG,<ref name=omim165080>{{OMIM\|165080}}</ref> ETV1, E1AF and FEV.
			*Lack of molecular findings does ''not'' exclude Ewing sarcoma.
			*Testing:
			**A break apart probe for EWS is a common way to look for pathologic change, as it covers almost all variants.

			==Electron microscopy==
			*Primitive cell junctions.
			*Clear zone (glycogen lakes).

			==See also==
			*[[Bone]].
			*[[Chondro-osseous tumours]].

			==References==
			{{Reflist\|2}}

	[[Category:Diagnosis]]		[[Category:Diagnosis]]
			[[Category:Chondro-osseous tumours]]

Michael: +cat.

2011-10-20T04:34:40Z

+cat.

← Older revision		Revision as of 04:34, 20 October 2011
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	#redirect [[Chondro-osseous tumours#Ewing sarcoma]]		#redirect [[Chondro-osseous tumours#Ewing sarcoma]]

			[[Category:Diagnosis]]