Difference between revisions of "Embryonal tumour with multilayered rosettes"
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**Embryonal tumour with multilayered rosettes, NOS. | **Embryonal tumour with multilayered rosettes, NOS. | ||
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> | |||
==Clinical presentation== | ==Clinical presentation== | ||
* Usu. age <4 years. | * Usu. age <4 years. | ||
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*LIN28+ve. | *LIN28+ve. | ||
<gallery>File:LIN28 ependymoblastoma.jpg| LIN28 positive ETMR.</gallery> | <gallery>File:LIN28 ependymoblastoma.jpg| LIN28 positive ETMR.</gallery> | ||
==See also== | |||
*[[Embryonal tumour with abundant neuropil and true rosettes]]. | |||
==References== | |||
{{Reflist|1}} | |||
[[Category:Diagnosis]] | |||
[[Category:Neuropathology]] | |||
Revision as of 16:36, 29 September 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:
- Embryonal tumour with abundant neuropil and true rosettes is considered a subset of this.[1]
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
- ↑ 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.