Difference between revisions of "Embryonal tumour with abundant neuropil and true rosettes"
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'''Embryonal tumour with abundant neuropil and true rosettes''', abbreviated '''ETANTR''', is a very rare [[neuropathology]] tumour reported only in children. It is currently not listed in the WHO CNS tumour classification. There is consens, to group this entity together with ependymoblastomas under the umbrella term [[embryonal tumour with multilayered rosettes]] (ETMR).<ref name=pmid20532895>{{Cite journal | last1 = Paulus | first1 = W. | last2 = Kleihues | first2 = P. | title = Genetic profiling of CNS tumors extends histological classification. | journal = Acta Neuropathol | volume = 120 | issue = 2 | pages = 269-70 | month = Aug | year = 2010 | doi = 10.1007/s00401-010-0710-1 | PMID = 20532895 }}</ref> | |||
==General== | |||
*Super rare. | |||
*Reported only in children <4 years old.<ref name=pmid16551982/> | |||
* First description in 2000 by Charles Eberhart. <ref>{{Cite journal | last1 = Eberhart | first1 = CG. | last2 = Brat | first2 = DJ. | last3 = Cohen | first3 = KJ. | last4 = Burger | first4 = PC. | title = Pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes. | journal = Pediatr Dev Pathol | volume = 3 | issue = 4 | pages = 346-52 | month = | year = | doi = | PMID = 10890250 }}</ref> | |||
==Microscopic== | |||
Features:<ref name=pmid20499240>{{Cite journal | last1 = Ferri Niguez | first1 = B. | last2 = Martínez-Lage | first2 = JF. | last3 = Almagro | first3 = MJ. | last4 = Fuster | first4 = JL. | last5 = Serrano | first5 = C. | last6 = Torroba | first6 = MA. | last7 = Sola | first7 = J. | title = Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update. | journal = Childs Nerv Syst | volume = 26 | issue = 8 | pages = 1003-8 | month = Aug | year = 2010 | doi = 10.1007/s00381-010-1179-x | PMID = 20499240 }}</ref> | |||
*[[Small round blue cell tumour]]. | |||
*True rosettes = flower-like cluster of cells that surrounds a space.<ref name=pmid16551982>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref> | |||
*Fibrillary neuropil. | |||
**Meshwork of fibers. | |||
DDx: | |||
* CNS [[Primitive neuroectodermal tumour]]. | |||
*Desmoplastic [[Medulloblastoma]]. | |||
===Images=== | |||
*[http://path.upmc.edu/cases/case585.html ETANTR - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case666.html ETANTR - another case (upmc.edu)]. | |||
==See also== | |||
*[[Neuropathology tumours]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology tumours]] | |||
Latest revision as of 17:16, 29 September 2017
Embryonal tumour with abundant neuropil and true rosettes, abbreviated ETANTR, is a very rare neuropathology tumour reported only in children. It is currently not listed in the WHO CNS tumour classification. There is consens, to group this entity together with ependymoblastomas under the umbrella term embryonal tumour with multilayered rosettes (ETMR).[1]
General
- Super rare.
- Reported only in children <4 years old.[2]
- First description in 2000 by Charles Eberhart. [3]
Microscopic
Features:[4]
- Small round blue cell tumour.
- True rosettes = flower-like cluster of cells that surrounds a space.[2]
- Fibrillary neuropil.
- Meshwork of fibers.
DDx:
- CNS Primitive neuroectodermal tumour.
- Desmoplastic Medulloblastoma.
Images
See also
References
- ↑ Paulus, W.; Kleihues, P. (Aug 2010). "Genetic profiling of CNS tumors extends histological classification.". Acta Neuropathol 120 (2): 269-70. doi:10.1007/s00401-010-0710-1. PMID 20532895.
- ↑ 2.0 2.1 Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
- ↑ Eberhart, CG.; Brat, DJ.; Cohen, KJ.; Burger, PC.. "Pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes.". Pediatr Dev Pathol 3 (4): 346-52. PMID 10890250.
- ↑ Ferri Niguez, B.; Martínez-Lage, JF.; Almagro, MJ.; Fuster, JL.; Serrano, C.; Torroba, MA.; Sola, J. (Aug 2010). "Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.". Childs Nerv Syst 26 (8): 1003-8. doi:10.1007/s00381-010-1179-x. PMID 20499240.