Embryonal tumour with multilayered rosettes

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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.

Microscopy

  • Rosettes (often multilayered).
  • Small cells.
  • Fibrillar zones (neuropil-like areas).
  • Neoplastic ganglion cells.
  • Papillar and tubular growth (primitive neural tubes).
    • PAS-positive membranes.
  • Glial maturation after treatment (rare).

IHC

  • LIN28+ve.
    • Note: Some AT/RT may be focally +ve. [3]
  • CD99: focally +ve.
  • Synaptophysin: Neuropil-like areas +ve.
  • GFAP: usu -ve.
  • INI1 +ve.
  • Mib1: 20-80%.

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.
  3. Rao, S.; Rajeswarie, RT.; Chickabasaviah Yasha, T.; Nandeesh, BN.; Arivazhagan, A.; Santosh, V. (Jul 2017). "LIN28A, a sensitive immunohistochemical marker for Embryonal Tumor with Multilayered Rosettes (ETMR), is also positive in a subset of Atypical Teratoid/Rhabdoid Tumor (AT/RT).". Childs Nerv Syst. doi:10.1007/s00381-017-3551-6. PMID 28744687.