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.
Note: ETMR is an umbrella term for following subsets:
- Embryonal tumour with abundant neuropil and true rosettes [1]
- Ependymoblastoma [2]
- Medulloepithelioma
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%.
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.
- ↑ 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.
- ↑ 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.