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.

Rosette

A halo or spoke-wheel arrangement of cells surrounding a central core or hub. The central hub may consist of an empty-appearing lumen or a space filled with cytoplasmic processes. The cytoplasm of each of the cells in the rosette is often wedge-shaped with the apex directed toward the central core; the nuclei of the cells participating in the rosette are peripherally positioned and form a ring or halo around the hub.

Total: 22

(per page)

PMID (PMCID)
29432310	FEMALE	Infant, Newborn
	A Congenital Case of Ependymoblastoma: A Rare and Aggressive Brain Tumor.
	Grassham CD, Rady S, Foster K, SantaCruz KS, Kuttesch JF, Maxwell JR. J Pediatr Hematol Oncol. 2019;41(1):e34-e37.
	This report of a congenital ependymoblastoma diagnosed at birth aims to highlight how much remains unknown about embryonal tumor with multilayered rosettes and the devastating prognosis of this condition.
29432310	FEMALE	Infant, Newborn
	A Congenital Case of Ependymoblastoma: A Rare and Aggressive Brain Tumor.
	Grassham CD, Rady S, Foster K, SantaCruz KS, Kuttesch JF, Maxwell JR. J Pediatr Hematol Oncol. 2019;41(1):e34-e37.
	Of these, ependymoblastoma is a profoundly aggressive embryonal brain tumor that is included in the diagnostic entity known as an embryonal tumor with multilayered rosettes.
26658063	MALE	Infant
	Embryonal Tumor With Abundant Neuropil and True Rosettes: A Distinct Immunohistochemical Pattern.
	Al-Salam S, Al Alashari M. Appl Immunohistochem Mol Morphol. 2016;24(6):e41-9.
	Embryonal tumors with abundant neuropil and true rosettes (ETANTR) are rare pediatric embryonal neoplasms that combine features of neuroblastoma and ependymoblastoma.
26252622	OTHER
	Embryonal tumor with multilayered rosettes of the fourth ventricle: case report.
	Edmonson CA, Weaver KJ, Kresak J, Pincus DW. J Neurosurg Pediatr. 2015;16(5):579-583.
	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.
26299744	MALE
	[Embryonal Tumors with Multilayer Rosettes--Rare Central Nervous System Tumors in Infants].
	Pleko M, Husakova K, Kaiserova E, Tichy M, Zamenik J. Klin Onkol. 2015;28(4):288-92.
	This group is composed of medulloepithelioma, ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes.
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.
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.
23865520 (3867594)	FEMALE
	The role of CD133+ cells in a recurrent embryonal tumor with abundant neuropil and true rosettes (ETANTR).
	Hervey-Jumper SL, Altshuler DB, Wang AC, He X, Maher CO, Robertson PL, Garton HJ, Fan X, Muraszko KM, Camelo-Piragua S. Brain Pathol. 2014;24(1):45-51.
	Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands.
23865520 (3867594)	FEMALE
	The role of CD133+ cells in a recurrent embryonal tumor with abundant neuropil and true rosettes (ETANTR).
	Hervey-Jumper SL, Altshuler DB, Wang AC, He X, Maher CO, Robertson PL, Garton HJ, Fan X, Muraszko KM, Camelo-Piragua S. Brain Pathol. 2014;24(1):45-51.
	Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands.
23865520 (3867594)	FEMALE
	The role of CD133+ cells in a recurrent embryonal tumor with abundant neuropil and true rosettes (ETANTR).
	Hervey-Jumper SL, Altshuler DB, Wang AC, He X, Maher CO, Robertson PL, Garton HJ, Fan X, Muraszko KM, Camelo-Piragua S. Brain Pathol. 2014;24(1):45-51.
	Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a recently described embryonal neoplasm of the central nervous system, consisting of a well-circumscribed embryonal tumor of infancy with mixed features of ependymoblastoma (multilayer ependymoblastic rosettes and pseudorosettes) and neuroblastoma (neuroblastic rosettes) in the presence of neuropil-like islands.
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.
	All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils.
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.
	All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils.
23791209	FEMALE	Young Adult
	Anaplastic ependymoma with ependymoblastic multilayered rosettes.
	Nobusawa S, Suzuki A, Nagaishi M, Isoda K, Ikota H, Yokoo H, Hirato J, Nakazato Y. Hum Pathol. 2013;44(11):2597-602.
	Here, we report an autopsy case involving a 24-year-old woman with a frontal lobe tumor, which showed the aggregation of true rosettes with multilayering of tumor cells resembling the ependymoblastoma histology.
23791209	FEMALE	Young Adult
	Anaplastic ependymoma with ependymoblastic multilayered rosettes.
	Nobusawa S, Suzuki A, Nagaishi M, Isoda K, Ikota H, Yokoo H, Hirato J, Nakazato Y. Hum Pathol. 2013;44(11):2597-602.
	Ependymal rosettes, forming a single layer of tumor cells, differ from ependymoblastic multilayered rosettes, which are characteristic histologic features of ependymoblastoma, a variant of central nervous system primitive neuroectodermal tumor.
23791209	FEMALE	Young Adult
	Anaplastic ependymoma with ependymoblastic multilayered rosettes.
	Nobusawa S, Suzuki A, Nagaishi M, Isoda K, Ikota H, Yokoo H, Hirato J, Nakazato Y. Hum Pathol. 2013;44(11):2597-602.
	Ependymal rosettes, forming a single layer of tumor cells, differ from ependymoblastic multilayered rosettes, which are characteristic histologic features of ependymoblastoma, a variant of central nervous system primitive neuroectodermal tumor.
23791209	FEMALE	Young Adult
	Anaplastic ependymoma with ependymoblastic multilayered rosettes.
	Nobusawa S, Suzuki A, Nagaishi M, Isoda K, Ikota H, Yokoo H, Hirato J, Nakazato Y. Hum Pathol. 2013;44(11):2597-602.
	Molecular and cytogenetic analyses revealed the absence of 19q13.42 amplification, a specific molecular hallmark of ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes, supporting the diagnosis of anaplastic ependymoma.
24558755	MALE	Infant
	[Are the medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes the same entity?].
	Ryzhova MV, Kushel' IuV, Zheludkova OG, Shishkina LV, Panina TN, Melikian AG, Zemlianskii MIu, Lemeneva NV. Zh Vopr Neirokhir Im N N Burdenko. 2013;77(6):51-5.
	[Are the medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes the same entity?
24558755	MALE	Infant
	[Are the medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes the same entity?].
	Ryzhova MV, Kushel' IuV, Zheludkova OG, Shishkina LV, Panina TN, Melikian AG, Zemlianskii MIu, Lemeneva NV. Zh Vopr Neirokhir Im N N Burdenko. 2013;77(6):51-5.
	Presence of these both aberrations suggesting that medulloepithelioma, ependymoblastoma and embryonal tumor with multilayered rosettes are the same entity.
21481009	MALE
	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.
	Wang Y, Chu SG, Xiong J, Cheng HX, Chen H, Yao XH. Neuropathology. 2011;31(6):639-47.
	Recently, the term "embryonal tumor with multilayered rosettes" (ETMR), including embryonal tumor with abundant neuropil and true rosettes (ETANTR) and ependymoblastoma (EBL) as a distinct tumor entity, has become an important topic of discussion for neuropathologists since the discovery of a unique genomic alteration in 2009.
21481009	MALE
	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.
	Wang Y, Chu SG, Xiong J, Cheng HX, Chen H, Yao XH. Neuropathology. 2011;31(6):639-47.
	Recently, the term "embryonal tumor with multilayered rosettes" (ETMR), including embryonal tumor with abundant neuropil and true rosettes (ETANTR) and ependymoblastoma (EBL) as a distinct tumor entity, has become an important topic of discussion for neuropathologists since the discovery of a unique genomic alteration in 2009.