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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.
 
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

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
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
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

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
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
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
Cenacchi G, Giangaspero F
J Neuropathol Exp Neurol 2004 Mar;63(3):185-92. doi: 10.1093/jnen/63.3.185. PMID: 15055442
McComb RD, Burger PC
Neurol Clin 1985 Nov;3(4):711-28. PMID: 3001488

Diagnosis

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
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
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
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
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

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
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
Sehgal SN
Transplant Proc 2003 May;35(3 Suppl):7S-14S. doi: 10.1016/s0041-1345(03)00211-2. PMID: 12742462
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
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

Prognosis

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
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
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
MacDonald TJ
J Child Neurol 2008 Oct;23(10):1195-204. doi: 10.1177/0883073808321769. PMID: 18952586Free PMC Article
McComb RD, Burger PC
Neurol Clin 1985 Nov;3(4):711-28. PMID: 3001488

Clinical prediction guides

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
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
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
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
Hirato J, Nakazato Y, Ogawa A
Clin Neuropathol 1994 Jan-Feb;13(1):1-11. PMID: 7518371

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