Embryonal tumour with multilayered rosettes - Revision history

Jensflorian: /* IHC */ molecular update

2019-01-23T09:09:36Z

IHC: molecular update

← Older revision		Revision as of 09:09, 23 January 2019
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	File:MIB1 ependymoblastoma.jpg \| MIB1 in ETMR rosettes.		File:MIB1 ependymoblastoma.jpg \| MIB1 in ETMR rosettes.
	</gallery>		</gallery>

			==Molecular==
			*Chr 2 gain.
			*C19MC amplification. <ref>{{Cite journal \| last1 = Spence \| first1 = T. \| last2 = Sin-Chan \| first2 = P. \| last3 = Picard \| first3 = D. \| last4 = Barszczyk \| first4 = M. \| last5 = Hoss \| first5 = K. \| last6 = Lu \| first6 = M. \| last7 = Kim \| first7 = SK. \| last8 = Ra \| first8 = YS. \| last9 = Nakamura \| first9 = H. \| title = CNS-PNETs with C19MC amplification and/or LIN28 expression comprise a distinct histogenetic diagnostic and therapeutic entity. \| journal = Acta Neuropathol \| volume = 128 \| issue = 2 \| pages = 291-303 \| month = Aug \| year = 2014 \| doi = 10.1007/s00401-014-1291-1 \| PMID = 24839957 }}</ref>
			*Some rare LIN28+ve cases without C19MC amplification may show DICER1 mutations.<ref>{{Cite journal \| last1 = Uro-Coste \| first1 = E. \| last2 = Masliah-Planchon \| first2 = J. \| last3 = Siegfried \| first3 = A. \| last4 = Blanluet \| first4 = M. \| last5 = Lambo \| first5 = S. \| last6 = Kool \| first6 = M. \| last7 = Roujeau \| first7 = T. \| last8 = Boetto \| first8 = S. \| last9 = Palenzuela \| first9 = G. \| title = ETMR-like infantile cerebellar embryonal tumors in the extended morphologic spectrum of DICER1-related tumors. \| journal = Acta Neuropathol \| volume = 137 \| issue = 1 \| pages = 175-177 \| month = Jan \| year = 2019 \| doi = 10.1007/s00401-018-1935-7 \| PMID = 30446821 }}</ref>

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

Jensflorian: cat

2017-10-04T12:55:33Z

cat

← Older revision		Revision as of 12:55, 4 October 2017
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	[[Category:Diagnosis]]		[[Category:Diagnosis]]
	[[Category:Neuropathology]]		[[Category:Neuropathology tumours]]

Jensflorian: DDx

2017-10-04T12:54:42Z

DDx

← Older revision		Revision as of 12:54, 4 October 2017
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	File:Neuroblasts ependymoblastoma.jpg \| ETMR, ganglion cells.		File:Neuroblasts ependymoblastoma.jpg \| ETMR, ganglion cells.
	</gallery>		</gallery>

			DDx:
			*[[AT/RT]].
			*[[Medulloblastoma]].
			*[[Ependymoma]].
			* CNS embryonal tumor, NOS.
			* Pediatric [[glioblastoma]].

	==IHC==		==IHC==

Jensflorian: WHO

2017-10-04T12:07:18Z

WHO

← Older revision		Revision as of 12:07, 4 October 2017
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	==General==		==General==
	* Extremely rare.		* Extremely rare.
			* Histologically WHO grade IV tumor.
	* ETMR historically had been termed CNS PNET.		* ETMR historically had been termed CNS PNET.
	* The WHO2016 CNS classification contains two groups:		* The WHO2016 CNS classification contains two groups:
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	*[[Embryonal tumour with abundant neuropil and true rosettes]] <ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>		*[[Embryonal tumour with abundant neuropil and true rosettes]] <ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>
	[[Ependymoblastoma]] <ref name=pmid19120373>{{Cite journal \| last1 = Judkins \| first1 = AR. \| last2 = Ellison \| first2 = DW. \| title = Ependymoblastoma: dear, damned, distracting diagnosis, farewell!. \| journal = Brain Pathol \| volume = 20 \| issue = 1 \| pages = 133-9 \| month = Jan \| year = 2010 \| doi = 10.1111/j.1750-3639.2008.00253.x \| PMID = 19120373 }}</ref>		[[Ependymoblastoma]] <ref name=pmid19120373>{{Cite journal \| last1 = Judkins \| first1 = AR. \| last2 = Ellison \| first2 = DW. \| title = Ependymoblastoma: dear, damned, distracting diagnosis, farewell!. \| journal = Brain Pathol \| volume = 20 \| issue = 1 \| pages = 133-9 \| month = Jan \| year = 2010 \| doi = 10.1111/j.1750-3639.2008.00253.x \| PMID = 19120373 }}</ref>
	*[[Medulloepithelioma]]<ref>{{Cite journal \| last1 = Korshunov \| first1 = A. \| last2 = Jakobiec \| first2 = FA. \| last3 = Eberhart \| first3 = CG. \| last4 = Hovestadt \| first4 = V. \| last5 = Capper \| first5 = D. \| last6 = Jones \| first6 = DT. \| last7 = Sturm \| first7 = D. \| last8 = Stagner \| first8 = AM. \| last9 = Edward \| first9 = DP. \| title = Comparative integrated molecular analysis of intraocular medulloepitheliomas and central nervous system embryonal tumors with multilayered rosettes confirms that they are distinct nosologic entities. \| journal = Neuropathology \| volume = 35 \| issue = 6 \| pages = 538-44 \| month = Dec \| year = 2015 \| doi = 10.1111/neup.12227 \| PMID = 26183384 }}</ref>		*[[Medulloepithelioma]] <ref>{{Cite journal \| last1 = Korshunov \| first1 = A. \| last2 = Jakobiec \| first2 = FA. \| last3 = Eberhart \| first3 = CG. \| last4 = Hovestadt \| first4 = V. \| last5 = Capper \| first5 = D. \| last6 = Jones \| first6 = DT. \| last7 = Sturm \| first7 = D. \| last8 = Stagner \| first8 = AM. \| last9 = Edward \| first9 = DP. \| title = Comparative integrated molecular analysis of intraocular medulloepitheliomas and central nervous system embryonal tumors with multilayered rosettes confirms that they are distinct nosologic entities. \| journal = Neuropathology \| volume = 35 \| issue = 6 \| pages = 538-44 \| month = Dec \| year = 2015 \| doi = 10.1111/neup.12227 \| PMID = 26183384 }}</ref>

Jensflorian: /* Microscopy */ +images

2017-10-04T12:05:35Z

Microscopy: +images

← Older revision		Revision as of 12:05, 4 October 2017
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	File:Ependymoblastoma.jpg \| ETMR, ependymoblastoma type.		File:Ependymoblastoma.jpg \| ETMR, ependymoblastoma type.
	File:Ependymoblastoma ETMRjpg.jpg \| ETMR, poorly undifferentiated cells.		File:Ependymoblastoma ETMRjpg.jpg \| ETMR, poorly undifferentiated cells.
			File:Ependymoblastoma-Histology.jpg \| ETMR, rosettes.
	File:Neuroblasts ependymoblastoma.jpg \| ETMR, ganglion cells.		File:Neuroblasts ependymoblastoma.jpg \| ETMR, ganglion cells.
			</gallery>

	==IHC==		==IHC==
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	*Mib1: 20-80%.		*Mib1: 20-80%.

	<gallery>File:LIN28 ependymoblastoma.jpg\| LIN28 positive ETMR.</gallery>		<gallery>
			File:LIN28 ependymoblastoma.jpg\| LIN28 positive ETMR.
			File:MIB1 ependymoblastoma.jpg \| MIB1 in ETMR rosettes.
			</gallery>

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

Jensflorian: /* Microscopy */ +images

2017-10-04T12:04:02Z

Microscopy: +images

← Older revision		Revision as of 12:04, 4 October 2017
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	* Papillar and tubular growth (primitive neural tubes).		* Papillar and tubular growth (primitive neural tubes).
	**PAS-positive membranes.		**PAS-positive membranes.
	* Glial maturation after treatment (rare).		* Glial/neuronal maturation after treatment (rare).
			<gallery>
			File:Medulloepithelioma Histology.jpg \| ETMR, medulloepithelioma type.
			File:Ependymoblastoma.jpg \| ETMR, ependymoblastoma type.
			File:Ependymoblastoma ETMRjpg.jpg \| ETMR, poorly undifferentiated cells.
			File:Neuroblasts ependymoblastoma.jpg \| ETMR, ganglion cells.

	==IHC==		==IHC==

Jensflorian: see also

2017-10-04T11:55:43Z

see also

← Older revision		Revision as of 11:55, 4 October 2017
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	Note:		Note:
	ETMR is an umbrella term for ~~following subsets~~:		ETMR is an umbrella term for tumors formerly known as:<ref>{{Cite journal \| last1 = Korshunov \| first1 = A. \| last2 = Sturm \| first2 = D. \| last3 = Ryzhova \| first3 = M. \| last4 = Hovestadt \| first4 = V. \| last5 = Gessi \| first5 = M. \| last6 = Jones \| first6 = DT. \| last7 = Remke \| first7 = M. \| last8 = Northcott \| first8 = P. \| last9 = Perry \| first9 = A. \| title = Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. \| journal = Acta Neuropathol \| volume = 128 \| issue = 2 \| pages = 279-89 \| month = Aug \| year = 2014 \| doi = 10.1007/s00401-013-1228-0 \| PMID = 24337497 }}</ref>

	*[[Embryonal tumour with abundant neuropil and true rosettes]] <ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>		*[[Embryonal tumour with abundant neuropil and true rosettes]] <ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>
	[[Ependymoblastoma]] <ref name=pmid19120373>{{Cite journal \| last1 = Judkins \| first1 = AR. \| last2 = Ellison \| first2 = DW. \| title = Ependymoblastoma: dear, damned, distracting diagnosis, farewell!. \| journal = Brain Pathol \| volume = 20 \| issue = 1 \| pages = 133-9 \| month = Jan \| year = 2010 \| doi = 10.1111/j.1750-3639.2008.00253.x \| PMID = 19120373 }}</ref>		[[Ependymoblastoma]] <ref name=pmid19120373>{{Cite journal \| last1 = Judkins \| first1 = AR. \| last2 = Ellison \| first2 = DW. \| title = Ependymoblastoma: dear, damned, distracting diagnosis, farewell!. \| journal = Brain Pathol \| volume = 20 \| issue = 1 \| pages = 133-9 \| month = Jan \| year = 2010 \| doi = 10.1111/j.1750-3639.2008.00253.x \| PMID = 19120373 }}</ref>
	*[[Medulloepithelioma]]		*[[Medulloepithelioma]]<ref>{{Cite journal \| last1 = Korshunov \| first1 = A. \| last2 = Jakobiec \| first2 = FA. \| last3 = Eberhart \| first3 = CG. \| last4 = Hovestadt \| first4 = V. \| last5 = Capper \| first5 = D. \| last6 = Jones \| first6 = DT. \| last7 = Sturm \| first7 = D. \| last8 = Stagner \| first8 = AM. \| last9 = Edward \| first9 = DP. \| title = Comparative integrated molecular analysis of intraocular medulloepitheliomas and central nervous system embryonal tumors with multilayered rosettes confirms that they are distinct nosologic entities. \| journal = Neuropathology \| volume = 35 \| issue = 6 \| pages = 538-44 \| month = Dec \| year = 2015 \| doi = 10.1111/neup.12227 \| PMID = 26183384 }}</ref>



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	==See also==		==See also==
	*[[Embryonal tumour with abundant neuropil and true rosettes]].		*[[Embryonal tumour with abundant neuropil and true rosettes]].
			*[[Ependymoblastoma]].
			*[[Medulloepithelioma]].

	==References==		==References==

Jensflorian: +histo

2017-10-04T11:52:01Z

+histo

← Older revision		Revision as of 11:52, 4 October 2017
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	* Rarely cysts, calcifications.		* Rarely cysts, calcifications.
	* Widespread infiltration.		* Widespread infiltration.

			==Microscopy==
			* Rosettes (often multilayered).
			* Small cells.
			* Fibrillar zones (neuropil-like areas).
			* Neoplastic ganglion cells.
			* Papillar and tubular growth (primitive neural tubes).
			**PAS-positive membranes.
			* Glial maturation after treatment (rare).

	==IHC==		==IHC==

Jensflorian: IHC

2017-10-04T11:45:15Z

IHC

← Older revision		Revision as of 11:45, 4 October 2017
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	==IHC==		==IHC==
	*LIN28+ve.		*LIN28+ve.
			**Note: Some AT/RT may be focally +ve. <ref>{{Cite journal \| last1 = Rao \| first1 = S. \| last2 = Rajeswarie \| first2 = RT. \| last3 = Chickabasaviah Yasha \| first3 = T. \| last4 = Nandeesh \| first4 = BN. \| last5 = Arivazhagan \| first5 = A. \| last6 = Santosh \| first6 = V. \| title = LIN28A, a sensitive immunohistochemical marker for Embryonal Tumor with Multilayered Rosettes (ETMR), is also positive in a subset of Atypical Teratoid/Rhabdoid Tumor (AT/RT). \| journal = Childs Nerv Syst \| volume = \| issue = \| pages = \| month = Jul \| year = 2017 \| doi = 10.1007/s00381-017-3551-6 \| PMID = 28744687 }}</ref>
			*CD99: focally +ve.
			*Synaptophysin: Neuropil-like areas +ve.
			*GFAP: usu -ve.
			*INI1 +ve.
			*Mib1: 20-80%.

	<gallery>File:LIN28 ependymoblastoma.jpg\| LIN28 positive ETMR.</gallery>		<gallery>File:LIN28 ependymoblastoma.jpg\| LIN28 positive ETMR.</gallery>

Jensflorian: ETMR subsetzs

2017-10-04T11:39:24Z

ETMR subsetzs

← Older revision		Revision as of 11:39, 4 October 2017
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	Note:		Note:
	*[[Embryonal tumour with abundant neuropil and true rosettes]] ~~is considered a subset of this.~~<ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>		ETMR is an umbrella term for following subsets:
			*[[Embryonal tumour with abundant neuropil and true rosettes]] <ref name=pmid23863344>{{Cite journal \| last1 = Ceccom \| first1 = J. \| last2 = Bourdeaut \| first2 = F. \| last3 = Loukh \| first3 = N. \| last4 = Rigau \| first4 = V. \| last5 = Milin \| first5 = S. \| last6 = Takin \| first6 = R. \| last7 = Richer \| first7 = W. \| last8 = Uro-Coste \| first8 = E. \| last9 = Couturier \| first9 = J. \| title = Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature. \| journal = Clin Neuropathol \| volume = 33 \| issue = 1 \| pages = 15-22 \| month = \| year = \| doi = 10.5414/NP300636 \| PMID = 23863344 }}</ref>
			[[Ependymoblastoma]] <ref name=pmid19120373>{{Cite journal \| last1 = Judkins \| first1 = AR. \| last2 = Ellison \| first2 = DW. \| title = Ependymoblastoma: dear, damned, distracting diagnosis, farewell!. \| journal = Brain Pathol \| volume = 20 \| issue = 1 \| pages = 133-9 \| month = Jan \| year = 2010 \| doi = 10.1111/j.1750-3639.2008.00253.x \| PMID = 19120373 }}</ref>
			*[[Medulloepithelioma]]


	==Clinical presentation==		==Clinical presentation==
	* Usu. age <4 years.		* Usu. age <4 years.