Difference between revisions of "Embryonal tumour with multilayered rosettes"

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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.

Revision as of 11:39, 4 October 2017

Embryonal tumour with multilayered rosettes, abbreviated ETMR, is a very rare neuropathology embryonal tumour with aggressive behaviour.

General

  • Extremely rare.
  • ETMR historically had been termed CNS PNET.
  • The WHO2016 CNS classification contains two groups:
    • Embryonal tumour with multilayered rosettes, C19MC-altered.
    • Embryonal tumour with multilayered rosettes, NOS.

Note: ETMR is an umbrella term for following subsets:


Clinical presentation

  • Usu. age <4 years.
  • 70% supratentorial, 30% infratentorial.
  • Raised intracranial pressure.

Imaging

  • Usu. enhancing.
  • Rarely cysts, calcifications.
  • Widespread infiltration.

IHC

  • LIN28+ve.

  • LIN28 positive ETMR.

See also

References

  1. ↑ Ceccom, J.; Bourdeaut, F.; Loukh, N.; Rigau, V.; Milin, S.; Takin, R.; Richer, W.; Uro-Coste, E. et al. "Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature.". Clin Neuropathol 33 (1): 15-22. doi:10.5414/NP300636. PMID 23863344.
  2. ↑ Judkins, AR.; Ellison, DW. (Jan 2010). "Ependymoblastoma: dear, damned, distracting diagnosis, farewell!*.". Brain Pathol 20 (1): 133-9. doi:10.1111/j.1750-3639.2008.00253.x. PMID 19120373.
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