A 3‐year‐ Old Girl With Altered Mental Status, Gait Difficulty, and Vomiting

Abstract

We report a case of a 3-year-old girl with a 9-month history of focal neurologic finding and signs of increased intracranial pressure. Radiological imaging studies showed a minimally heterogeneous enhancing large tumor involving left parietal region and partly filling the left lateral ventricle. Microscopically, the tumor contained prominent small true rosettes set into areas of fibrillar neuropil-like matrix. Foci of cellular tumor with extensive apoptosis were present. The neuropil-like areas and true rosettes were positive for synaptophysin immunostain. The tumor was negative for GFAP and neurofilament protein immunostains. MIB-1 proliferation index was high focally approaching 80%.

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 abundant neuropil and true rosettes (ETANTR) with a focal amplification at chromosome 19q13.42 locus: Further evidence of two new instances in China

“…first described a rare variant of pediatric neuroblastic brain tumors containing abundant neuropil and true rosettes (PNTANTR), which showed a distinctive histopathology, overlapping neuroblastic components with advanced neuronal differentiation, including neurocytes, ganglion cells and neuropil‐like background and ependymoblastic rosette formations, subsequently designated ETANTR, suggesting it represents a new clinicopathological entity 3,4 . This view has been supported by other researchers; 5,6,7,8,9,10,11,12 however, this tumor is not listed as a distinct entity in the 2007 WHO classification of embryonal tumors of the CNS 13 . On the other hand, EBL is categorized under CNS primitive neuroectodermal tumors (PNETs) characterized by the presence of ependymoblastic rosettes in association with undifferentiated small cells 13 .…”