Embryonal tumor with multilayered rosettes (ETMR)

Case contributed by Dr Hani Salam

Patient Data

Age: Young adult
Gender: Male

A large heterogeneous mass is present in the left parietal region with mass effect and surrounding edema. 

A large heterogeneous mass in the left parietal lobe is noted with prominent mass effect and central necrosis. 

Case Discussion

A young male patient with pathologically proven sPNET.

 

Editorial note: The age of the patient has not been provided, but looking at the dentition it appears to be a young adult. The diagnosis of supratentorial PNET/embryonal tumors with multilayered rosettes (ETMR) is rare outside of the first decade. As the saying goes "extraordinary claims require extraordinary proof" and as such, without more supporting evidence, the diagnostic certainty has been reduced to "possible". 

In an adult, it is probably more likely that this represents as glioblastoma with primitive neuronal component, possibly arising from an underlying lower grade tumor. 

Editorial note: The current (2016) WHO classification of CNS tumors has made substantial changes to tumors previously considered to be 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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Case information

rID: 8931
Published: 10th Mar 2010
Last edited: 13th Aug 2019
Inclusion in quiz mode: Included

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