Embryonal tumour with multilayered rosettes (ETMR)

Case contributed by Dr Jeremy Jones

Presentation

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).

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Case information

rID: 8083
Case created: 9th Jan 2010
Last edited: 26th May 2017
Inclusion in quiz mode: Included

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