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Ependymoblastoma

MedGen UID:
152150
•Concept ID:
C0700367
•
Neoplastic Process
Synonym: Neuroectodermal tumors primitive
SNOMED CT: Ependymoblastoma (715901002); Ependymoblastoma (21589007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0030066
Monarch Initiative: MONDO:0016715
Orphanet: ORPHA251880

Definition

A highly malignant embryonal tumor of infancy and young childhood characterized by neuroectodermal elements organized in distinctive multilayered rosettes. Ependymoblastomas are large lesions that occur in the supratentorial compartment, typically displaying a physical connection to the ventricular system. [from HPO]

Professional guidelines

PubMed

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179
Management and outcome of children and adolescents with non-medulloblastoma CNS embryonal tumors in Spain: room for improvement in standards of care.
de Rojas T, Bautista F, Flores M, Igual L, Rubio R, Bardón E, Navarro L, Murillo L, Hladun R, Cañete A, Garcia-Ariza M, Garrido C, Fernández-Teijeiro A, Quiroga E, Calvo C, Llort A, de Prada I, Madero L, Cruz O, Moreno L
J Neurooncol 2018 Mar;137(1):205-213. Epub 2017 Dec 16 doi: 10.1007/s11060-017-2713-4. PMID: 29248974
Evolving role of myeloablative chemotherapy in the treatment of childhood brain tumours.
Dallorso S, Dini G, Ladenstein R, Cama A, Milanaccio C, Barra S, Cappelli B, Garrè ML; EBMT-PDWP
Bone Marrow Transplant 2005 Mar;35 Suppl 1:S31-4. doi: 10.1038/sj.bmt.1704841. PMID: 15812527

Recent clinical studies

Etiology

Systematic comparison of MRI findings in pediatric ependymoblastoma with ependymoma and CNS primitive neuroectodermal tumor not otherwise specified.
Nowak J, Seidel C, Pietsch T, Alkonyi B, Fuss TL, Friedrich C, von Hoff K, Rutkowski S, Warmuth-Metz M
Neuro Oncol 2015 Aug;17(8):1157-65. Epub 2015 Apr 26 doi: 10.1093/neuonc/nov063. PMID: 25916887Free PMC Article
TP53, β-Catenin and c-myc/N-myc status in embryonal tumours with ependymoblastic rosettes.
Gessi M, Zur Muehlen A, Lauriola L, Gardiman MP, Giangaspero F, Pietsch T
Neuropathol Appl Neurobiol 2011 Jun;37(4):406-13. doi: 10.1111/j.1365-2990.2010.01151.x. PMID: 21073496
Molecular diagnostics of CNS embryonal tumors.
Pfister SM, Korshunov A, Kool M, Hasselblatt M, Eberhart C, Taylor MD
Acta Neuropathol 2010 Nov;120(5):553-66. Epub 2010 Sep 30 doi: 10.1007/s00401-010-0751-5. PMID: 20882288Free PMC Article
Evolving role of myeloablative chemotherapy in the treatment of childhood brain tumours.
Dallorso S, Dini G, Ladenstein R, Cama A, Milanaccio C, Barra S, Cappelli B, Garrè ML; EBMT-PDWP
Bone Marrow Transplant 2005 Mar;35 Suppl 1:S31-4. doi: 10.1038/sj.bmt.1704841. PMID: 15812527
Emerging tumor entities and variants of CNS neoplasms.
Cenacchi G, Giangaspero F
J Neuropathol Exp Neurol 2004 Mar;63(3):185-92. doi: 10.1093/jnen/63.3.185. PMID: 15055442

Diagnosis

Embryonal tumors with multilayered rosettes: A tertiary care centre experience.
Kumar N, Madan R, Gupta K, Chatterjee D, Uppal DK, Goyal S, Ballari N, Khosla D, Sahoo SK, Ahuja CK
Clin Neurol Neurosurg 2021 Mar;202:106508. Epub 2021 Jan 27 doi: 10.1016/j.clineuro.2021.106508. PMID: 33556852
Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179
MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases.
Nowak J, Seidel C, Berg F, Pietsch T, Friedrich C, von Hoff K, Rutkowski S, Warmuth-Metz M
AJNR Am J Neuroradiol 2014 Oct;35(10):1996-2001. Epub 2014 Jun 19 doi: 10.3174/ajnr.A4002. PMID: 24948504Free PMC Article
Ependymoblastoma with cystic change in a child.
Ding D, Zhao A, Qiu B, Xing D, Guan G, Guo Z
J Neurosurg Pediatr 2014 Jun;13(6):658-65. Epub 2014 Apr 11 doi: 10.3171/2014.3.PEDS13405. PMID: 24724717
Molecular diagnostics of CNS embryonal tumors.
Pfister SM, Korshunov A, Kool M, Hasselblatt M, Eberhart C, Taylor MD
Acta Neuropathol 2010 Nov;120(5):553-66. Epub 2010 Sep 30 doi: 10.1007/s00401-010-0751-5. PMID: 20882288Free PMC Article

Therapy

Evolving role of myeloablative chemotherapy in the treatment of childhood brain tumours.
Dallorso S, Dini G, Ladenstein R, Cama A, Milanaccio C, Barra S, Cappelli B, Garrè ML; EBMT-PDWP
Bone Marrow Transplant 2005 Mar;35 Suppl 1:S31-4. doi: 10.1038/sj.bmt.1704841. PMID: 15812527
Sirolimus: its discovery, biological properties, and mechanism of action.
Sehgal SN
Transplant Proc 2003 May;35(3 Suppl):7S-14S. doi: 10.1016/s0041-1345(03)00211-2. PMID: 12742462
Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group.
Robertson PL, Zeltzer PM, Boyett JM, Rorke LB, Allen JC, Geyer JR, Stanley P, Li H, Albright AL, McGuire-Cullen P, Finlay JL, Stevens KR Jr, Milstein JM, Packer RJ, Wisoff J
J Neurosurg 1998 Apr;88(4):695-703. doi: 10.3171/jns.1998.88.4.0695. PMID: 9525716
Clinical trials with the hexitol derivatives in the U.S.
Chiuten DF, Rozencweig M, Von Hoff DD, Muggia FM
Cancer 1981 Feb 1;47(3):442-51. doi: 10.1002/1097-0142(19810201)47:3<442::aid-cncr2820470304>3.0.co;2-1. PMID: 6784907
Dianhydrogalactitol and radiation therapy. Treatment of supratentorial glioma.
Eagan RT, Childs DS Jr, Layton DD Jr, Laws ER Jr, Bisel HF, Holbrook MA, Fleming TR
JAMA 1979 May 11;241(19):2046-50. PMID: 219269

Prognosis

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179
MRI characteristics of ependymoblastoma: results from 22 centrally reviewed cases.
Nowak J, Seidel C, Berg F, Pietsch T, Friedrich C, von Hoff K, Rutkowski S, Warmuth-Metz M
AJNR Am J Neuroradiol 2014 Oct;35(10):1996-2001. Epub 2014 Jun 19 doi: 10.3174/ajnr.A4002. PMID: 24948504Free PMC Article
Ependymoblastoma with cystic change in a child.
Ding D, Zhao A, Qiu B, Xing D, Guan G, Guo Z
J Neurosurg Pediatr 2014 Jun;13(6):658-65. Epub 2014 Apr 11 doi: 10.3171/2014.3.PEDS13405. PMID: 24724717
Molecular diagnostics of CNS embryonal tumors.
Pfister SM, Korshunov A, Kool M, Hasselblatt M, Eberhart C, Taylor MD
Acta Neuropathol 2010 Nov;120(5):553-66. Epub 2010 Sep 30 doi: 10.1007/s00401-010-0751-5. PMID: 20882288Free PMC Article
Aggressive infantile embryonal tumors.
MacDonald TJ
J Child Neurol 2008 Oct;23(10):1195-204. doi: 10.1177/0883073808321769. PMID: 18952586Free PMC Article

Clinical prediction guides

MYCN amplification predicts poor outcome for patients with supratentorial primitive neuroectodermal tumors of the central nervous system .
Gessi M, von Bueren A, Treszl A, zur Mühlen A, Hartmann W, Warmuth-Metz M, Rutkowski S, Pietsch T
Neuro Oncol 2014 Jul;16(7):924-32. doi: 10.1093/neuonc/not302. PMID: 24470553Free PMC Article
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 Jan-Feb;33(1):15-22. doi: 10.5414/NP300636. PMID: 23863344
Outcome of 11 children with ependymoblastoma treated within the prospective HIT-trials between 1991 and 2006.
Gerber NU, von Hoff K, von Bueren AO, Treulieb W, Warmuth-Metz M, Pietsch T, Soerensen N, Faldum A, Emser A, Schlegel PG, Deinlein F, Kortmann RD, Rutkowski S
J Neurooncol 2011 May;102(3):459-69. Epub 2011 Feb 11 doi: 10.1007/s11060-010-0347-x. PMID: 21308398
Tumor suppressor proteins are differentially affected in human ependymoblastoma and medulloblastoma cells exposed to nerve growth factor.
Antonelli A, Lenzi L, Nakagawara A, Osaki T, Chiaretti A, Aloe L
Cancer Invest 2007 Mar;25(2):94-101. doi: 10.1080/07357900701205689. PMID: 17453820
Expression of non-glial intermediate filament proteins in gliomas.
Hirato J, Nakazato Y, Ogawa A
Clin Neuropathol 1994 Jan-Feb;13(1):1-11. PMID: 7518371

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