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Embryonal tumor with multilayered rosettes (ETMR)

Case contributed by Jeremy Jones
Diagnosis almost certain

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-tumor edema 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 tumors has made substantial changes to tumors previously considered to be a supratentorial PNET, now classified as embryonal tumors 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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