Difference between revisions of "Embryonal tumour with multilayered rosettes"

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* Extremely rare.
* Extremely rare.
* ETMR historically had been termed CNS PNET.
* 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.
==Clinical presentation==
* Usu. age <4 years.
* 70% supratentorial, 30% infratentorial.
* Raised intracranial pressure.
==Imaging==
* Usu. enhancing.
* Rarely cysts, calcifications.
* Widespread infiltration.
==IHC==
*LIN28+ve.
<gallery>File:LIN28 ependymoblastoma.jpg| LIN28 positive ETMR.</gallery>

Revision as of 09:31, 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.

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.

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