Embryonal tumour with multilayered rosettes

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.