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Embryonal tumour with multilayered rosettes (view source)
Revision as of 09:09, 23 January 2019
, 09:09, 23 January 2019→IHC: molecular update
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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> | ||
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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> | |||
DDx: | |||
*[[AT/RT]]. | |||
*[[Medulloblastoma]]. | |||
*[[Ependymoma]]. | |||
* CNS embryonal tumor, NOS. | |||
* Pediatric [[glioblastoma]]. | |||
==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> | |||
==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== | ||
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[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology]] | [[Category:Neuropathology tumours]] | ||