, Volume 122, Issue 6, pp 783-785
Date: 28 Oct 2011

Multinodular leptomeningeal metastases from ETANTR contain both small blue cell and maturing neuropil elements

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Embryonal tumor with abundant neuropil and true rosettes (ETANTR), a rare, malignant neoplasm of infancy occurring in the supratentorial and infratentorial regions, is composed of a combination of small blue cells and maturing neuropil, sometimes with ganglion cell differentiation. True multilayered ependymoblastic rosettes (and occasionally Homer Wright neuroblastic rosettes) are present, and the recent work has demonstrated a unique focal genomic amplification at 19q13.42 as a signature genetic marker [9]. Prognosis is nearly universally poor, as was noted in the original description in 2000 by Eberhart et al. [4]. and in a recently updated series including the original 7 patients and 22 additional cases; 76% of patients died from their disease with a median survival of 9 months [7].

Interestingly, despite the adverse outcome, only 3 of 33 patients in that series [7], one reported by Ferri Niguez et al. [5], and “some” of the 16 patients in a series reported by Korshunov et al. [9], e ...