Embryonal tumour with multilayered rosettes (ETMR)
Child presenting with right sided neurological symptoms and a skull mass.
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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.
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).