Ependymoblastoma of the brainstem: MRI findings and differential diagnosis

Abstract

Ependymoblastoma (EBL) is a rare malignant CNS tumor of early childhood, listed as a subgroup of primitive neuroectodermal tumors (PNET) in the 2007 WHO Classification of Tumours of the Central Nervous System. Histologically, EBL can be defined by multilayered, mitotically active "ependymoblastic" rosettes with central lumen as a histological hallmark. The prognosis seems to be far inferior to other embryonal CNS tumors, and known clinical and MRI characteristics of EBL are based on scattered case reports. We present and discuss two uncommon cases of histopathologically confirmed ependymoblastoma that both seem to originate from the brainstem.

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] .…”

Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified

“…The findings for the 22 EBL cases have been recently described in greater detail. 14,15 Tables 1 and 2 demonstrate the analyzed imaging parameters and a summary of the results for each group. P-values are listed for each category.…”

“…The described MRI characteristics of EBL are based on the largest series comprising 22 centrally reviewed EBL cases, which have been recently described in detail by our group. 14,15 In contrast to the limited radiological data of EBL, imaging features of Nowak et al: MRI in ependymoblastoma, ependymoma, and CNS-PNET NOS EP are well documented in the literature, since EP represents the third most frequent intracranial neoplasm in children after pilocytic astrocytoma and medulloblastoma. 18 In our study, EBL occurred in very young children (mean age, 2.1 y) and with a male predisposition, whereas EP had a slightly (but significantly) higher age at diagnosis and girls were affected more frequently.…”

“…10 -13 We recently described the MRI characteristics of EBL, and our data suggest that it might share imaging features with CNS-PNET NOS. 14,15 However, a study that systematically compares imaging characteristics of EBL with other pediatric brain tumor entities has not yet been performed. With this study, we compare our recent collection of 22 EBLs with 22 cases of both EP and CNS-PNET NOS by paired analysis of defined MRI criteria.…”

Mri as a Tool of Differential Diagnostic in the Brainstem Damage in Children

“…У детей младше 3 лет могут возникать солидные примитивные нейроэктодермальные опухоли. Они фокально, экзофитно расположены обычно в мосту, умеренно накапливают контраст и часто распространяются субарахноидально [73][74][75]. Опухоли ствола у детей также могут развиваться при неврофиброматозе 1-го типа [76,77].…”