Embryonal tumor with multilayered rosettes (ETMR)

Discussion:

A biopsy of the central solid components was undertaken and revealed that the tumor was a supratentorial PNET. The biopsy of the lesion resulted in significant hemorrhage and 4 units of blood along with clotting factors were required.

Chemotherapy did not significantly alter the volume of MRI characteristics of the lesion and it continued to grow.

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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