Embryonal tumour with multilayered rosettes (ETMR)

Case contributed by Dr Jeremy Jones


Child presenting with right sided neurological symptoms and a skull mass.

Patient Data

Age: Child

Large heterogenous mass of the left cerebral hemisphere, centered in the frontoparietal region. It has cystic / necrotic components with extensive blooming on T2* weighted sequence consistent with blood products. Extensive vasogenic-tumour oedema spreads particularly anteriorly into the frontal lobe, with significant midline shift. 

The mass extends directly through the skull vault and into the subgaleal tissues. 

Case Discussion

Features are consistent with the subsequently confirmed supratentorial PNET.

Note: The current (2016) WHO classification of CNS tumours has made substantial changes to tumours previously considered to be a supratentorial PNET, now classified as embryonal tumours with multilayered rosettes (ETMR), along with a number of other entities, in recognition of characteristic amplification of the C19MC region on chromosome 19 (19q13.42).

PlayAdd to Share

Case information

rID: 8083
Published: 9th Jan 2010
Last edited: 16th Jul 2018
Inclusion in quiz mode: Included

Updating… Please wait.

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.