Ependymoblastoma

Ependymoblastoma is a rare type of primitive neuroectodermal tumor (PNET) that usually occurs in young children under the age of 2 and is histologically distinguished by the production of ependymoblastic rosettes. It is associated with an aggressive course and a poor prognosis.

Primitive neuroectodermal tumor

A tumor that originates in cells from the primitive neural crest. This group of tumors is characteirzed by the presence of primitive cells with elements of neuronal and/or glial differentiation.


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PMID (PMCID)
23863344
 FEMALE Infant
Embryonal tumor with multilayered rosettes: diagnostic tools update and review of the literature.
Ceccom J, Bourdeaut F, Loukh N, Rigau V, Milin S, Takin R, Richer W, Uro-Coste E, Couturier J, Bertozzi AI, Delattre O, Delisle MB.
Clin Neuropathol. 2014;33(1):15-22.
Embryonal tumor with multilayered rosettes (ETMR), including embryonal tumor with abundant neuropil and true rosettes (ETANTR), and ependymoblastoma (EBL) constitute a distinct entity of the primitive neuroectodermal tumor (PNET) family.
24464920
 MIXED_SAMPLE Infant
Ependymoblastoma of the brainstem: MRI findings and differential diagnosis.
Nowak J, Seidel C, Pietsch T, Friedrich C, von Hoff K, Rutkowski S, Warmuth-Metz M.
Pediatr Blood Cancer. 2014;61(6):1132-4.
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.
25208992
 MIXED_SAMPLE Infant
[Primitive neuroectodermal tumor of central nervous system with features of ependymoblastoma and neuroblastoma: a clinicopathologic study of 4 cases].
Wang R, Guan W, Wu X, Zhang W, Jiang B, Ma J, Wang L.
Zhonghua Bing Li Xue Za Zhi. 2014;43(6):403-7.
[Primitive neuroectodermal tumor of central nervous system with features of ependymoblastoma and neuroblastoma: a clinicopathologic study of 4 cases].
18097184
 MIXED_SAMPLE Infant, Newborn
Congenital brain tumors in a series of seven patients.
Nejat F, Kazmi SS, Ardakani SB.
Pediatr Neurosurg. 2008;44(1):1-8.
There were three teratomas, one primitive neuroectodermal tumor, one ependymoblastoma and one CPP.
7491192
 MALE Infant
Ependymoblastoma: MR presentation. A case report and review of the literature.
Dorsay TA, Rovira MJ, Ho VB, Kelley J.
Pediatr Radiol. 1995;25(6):433-5.
Ependymoblastoma is a rare and devastating tumor of childhood considered by most to be a subtype of primitive neuroectodermal tumors.