Embryonal tumor with multilayered rosettes (ETMR)

Discussion:

Given the supratentorial location and large size and heterogeneous appearance, in a young child, the favored diagnosis is a primitive neuroectodermal tumor of the CNS

Histology was told to confirm the diagnosis of a primitive neuroectodermal tumor of the CNS.

Note: The current (2016) WHO classification of CNS tumors has made substantial changes to tumors previously considered to be 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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