Published in:
01-12-2011 | Correspondence
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) with loss of morphological but retained genetic key features during progression
Authors:
Adelheid Woehrer, Irene Slavc, Andreas Peyrl, Thomas Czech, Christian Dorfer, Daniela Prayer, Susanne Stary, Berthold Streubel, Marina Ryzhova, Andrey Korshunov, Stefan M. Pfister, Christine Haberler
Published in:
Acta Neuropathologica
|
Issue 6/2011
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Excerpt
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently recognized, rare embryonal CNS tumor, which predominantly occurs in young children and is associated with a highly aggressive disease course [
1‐
3,
5‐
8,
13,
14,
18,
19,
23]. The histopathological diagnosis of ETANTR is based on the presence of primitive neuroectodermal tumor cells forming distinct multilayered ‘ependymoblastic’ rosettes and characteristic neuropil islands. Recently, genome-wide analyses have revealed a novel amplification at 19q13.42 [
16,
19], which is meanwhile considered the genetic hallmark of ETANTR [
13,
16]. The characteristic clinical, morphological, and genetic features support the concept of a distinct CNS PNET variant and suggest its introduction to the WHO classification of Tumors of the Central Nervous System [
17]. As the amplification at 19q13.42 has also been found in the vast majority of ependymoblastomas analyzed to date [
13,
16], the common genetic background suggests the fusion of these two tumor types to a single entity. …
