, Volume 122, Issue 6, pp 787-790
Date: 06 Nov 2011

Embryonal tumor with abundant neuropil and true rosettes (ETANTR) with loss of morphological but retained genetic key features during progression

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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 [13, 58, 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 fus