Embryonal tumor with multilayered rosettes of the fourth ventricle: case report

Abstract

<jats:p>Embryonal tumor with multilayered rosettes (ETMR) is a recently described pathological entity. These primitive central nervous system tumors harbor amplification of the 19q13.42 locus and resultant overexpression of the LIN28A protein. Although the WHO currently recognizes 3 distinct histopathological entities—embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma—recent studies indicate that these tumors have a common molecular profile and clinical course and that they are now classified as a single entity. Here the authors present a case of ETMR located in the fourth ventricle in a 12-month-old boy. The histopathology featured areas of neuropil-like stroma and highly cellular foci with characteristic multilayered rosettes. The authors discuss the clinical, radiological, and histopathological findings in this case and compare them with data in previously published cases in the literature. A review of studies assessing the molecular mechanisms underlying these tumors is also presented.</jats:p>

Radiation for ETMR: Literature review and case series of patients treated with proton therapy

“…Other authors have reviewed cases of MEP and EPL described since the 1920s to 1990 [45] , [49] , [50] , [51] . The patient characteristics, treatment, and outcome of these 204 cases plus the seven MD Anderson cases are summarized in Table 3 [1] , [5] , [7] , [9] , [14] , [49] , [50] , [51] , [52] , [53] , [54] , [55] , [56] , [57] , [58] , [59] , [60] , [61] , [62] , [63] , [64] , [65] , [66] , [67] , [68] , [69] , [70] , [71] , [72] , [73] , [74] , [75] , [76] , [77] , [78] , [79] , [80] , [81] , [82] , [83] , [84] , [85] , [86] , [87] , [88] , [89] , [90] , [91] , [92] , [93] , [94] , [95] , [96] , [97] , [98] .…”

Embryonal Tumor with Multilayered Rosettes, C19MC‐Altered: Clinical, Pathological, and Neuroimaging Findings

“…These tumors share a common molecular signature, C19MC alteration . Fewer than 100 cases of ETMR have been reported …”

Case report: Infratentorial Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR) in an 8-month-old Maine Coon

“…Human ETMR is an aggressive, WHO-grade IV tumor that occurs predominantly in infants under the age of 3 years ( 4 ). Nearly all tumors reside in the brain, ~70% occurring in supratentorial and 30% in infratentorial regions ( 12 , 23 ) with reports of ETMRs occurring in the fourth ventricle ( 32 ). Human ETMRs mostly present as large well-demarcated tumors.…”