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Pediatric cerebral ependymoblastoma

MedGen UID:
234125
Concept ID:
C1332962
Neoplastic Process
Synonyms: Childhood Cerebral Embryonal Tumor with Multilayered Rosettes, C19MC-Altered; childhood cerebral embryonal tumor with Multilayered Rosettes, C19MC-altered; childhood cerebral embryonal tumour with Multilayered Rosettes, C19MC-altered; Childhood Cerebral Ependymoblastoma; childhood cerebral ependymoblastoma; Pediatric Cerebral Ependymoblastoma; pediatric cerebral ependymoblastoma
 
Monarch Initiative: MONDO:0004378

Definition

An embryonal tumor with multilayered rosettes, C19MC-altered that arises from the cerebral hemispheres and occurs in children. [from NCI]

Professional guidelines

PubMed

Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Donovan LK, Delaidelli A, Joseph SK, Bielamowicz K, Fousek K, Holgado BL, Manno A, Srikanthan D, Gad AZ, Van Ommeren R, Przelicki D, Richman C, Ramaswamy V, Daniels C, Pallota JG, Douglas T, Joynt ACM, Haapasalo J, Nor C, Vladoiu MC, Kuzan-Fischer CM, Garzia L, Mack SC, Varadharajan S, Baker ML, Hendrikse L, Ly M, Kharas K, Balin P, Wu X, Qin L, Huang N, Stucklin AG, Morrissy AS, Cavalli FMG, Luu B, Suarez R, De Antonellis P, Michealraj A, Rastan A, Hegde M, Komosa M, Sirbu O, Kumar SA, Abdullaev Z, Faria CC, Yip S, Hukin J, Tabori U, Hawkins C, Aldape K, Daugaard M, Maris JM, Sorensen PH, Ahmed N, Taylor MD
Nat Med 2020 May;26(5):720-731. Epub 2020 Apr 27 doi: 10.1038/s41591-020-0827-2. PMID: 32341580Free PMC Article
Franceschi E, Hofer S, Brandes AA, Frappaz D, Kortmann RD, Bromberg J, Dangouloff-Ros V, Boddaert N, Hattingen E, Wiestler B, Clifford SC, Figarella-Branger D, Giangaspero F, Haberler C, Pietsch T, Pajtler KW, Pfister SM, Guzman R, Stummer W, Combs SE, Seidel C, Beier D, McCabe MG, Grotzer M, Laigle-Donadey F, Stücklin ASG, Idbaih A, Preusser M, van den Bent M, Weller M, Hau P
Lancet Oncol 2019 Dec;20(12):e715-e728. doi: 10.1016/S1470-2045(19)30669-2. PMID: 31797797

Recent clinical studies

Etiology

Jackson K, Packer RJ
Curr Neurol Neurosci Rep 2023 Dec;23(12):841-848. Epub 2023 Nov 9 doi: 10.1007/s11910-023-01316-9. PMID: 37943476Free PMC Article
Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Northcott PA, Robinson GW, Kratz CP, Mabbott DJ, Pomeroy SL, Clifford SC, Rutkowski S, Ellison DW, Malkin D, Taylor MD, Gajjar A, Pfister SM
Nat Rev Dis Primers 2019 Feb 14;5(1):11. doi: 10.1038/s41572-019-0063-6. PMID: 30765705
Cavalli FMG, Remke M, Rampasek L, Peacock J, Shih DJH, Luu B, Garzia L, Torchia J, Nor C, Morrissy AS, Agnihotri S, Thompson YY, Kuzan-Fischer CM, Farooq H, Isaev K, Daniels C, Cho BK, Kim SK, Wang KC, Lee JY, Grajkowska WA, Perek-Polnik M, Vasiljevic A, Faure-Conter C, Jouvet A, Giannini C, Nageswara Rao AA, Li KKW, Ng HK, Eberhart CG, Pollack IF, Hamilton RL, Gillespie GY, Olson JM, Leary S, Weiss WA, Lach B, Chambless LB, Thompson RC, Cooper MK, Vibhakar R, Hauser P, van Veelen MC, Kros JM, French PJ, Ra YS, Kumabe T, López-Aguilar E, Zitterbart K, Sterba J, Finocchiaro G, Massimino M, Van Meir EG, Osuka S, Shofuda T, Klekner A, Zollo M, Leonard JR, Rubin JB, Jabado N, Albrecht S, Mora J, Van Meter TE, Jung S, Moore AS, Hallahan AR, Chan JA, Tirapelli DPC, Carlotti CG, Fouladi M, Pimentel J, Faria CC, Saad AG, Massimi L, Liau LM, Wheeler H, Nakamura H, Elbabaa SK, Perezpeña-Diazconti M, Chico Ponce de León F, Robinson S, Zapotocky M, Lassaletta A, Huang A, Hawkins CE, Tabori U, Bouffet E, Bartels U, Dirks PB, Rutka JT, Bader GD, Reimand J, Goldenberg A, Ramaswamy V, Taylor MD
Cancer Cell 2017 Jun 12;31(6):737-754.e6. doi: 10.1016/j.ccell.2017.05.005. PMID: 28609654Free PMC Article
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article

Diagnosis

Rossor T, Yeh EA, Khakoo Y, Angelini P, Hemingway C, Irani SR, Schleiermacher G, Santosh P, Lotze T, Dale RC, Deiva K, Hero B, Klein A, de Alarcon P, Gorman MP, Mitchell WG, Lim M; OMS Study Group
Neurol Neuroimmunol Neuroinflamm 2022 May;9(3) Epub 2022 Mar 8 doi: 10.1212/NXI.0000000000001153. PMID: 35260471Free PMC Article
Franceschi E, Hofer S, Brandes AA, Frappaz D, Kortmann RD, Bromberg J, Dangouloff-Ros V, Boddaert N, Hattingen E, Wiestler B, Clifford SC, Figarella-Branger D, Giangaspero F, Haberler C, Pietsch T, Pajtler KW, Pfister SM, Guzman R, Stummer W, Combs SE, Seidel C, Beier D, McCabe MG, Grotzer M, Laigle-Donadey F, Stücklin ASG, Idbaih A, Preusser M, van den Bent M, Weller M, Hau P
Lancet Oncol 2019 Dec;20(12):e715-e728. doi: 10.1016/S1470-2045(19)30669-2. PMID: 31797797
Northcott PA, Robinson GW, Kratz CP, Mabbott DJ, Pomeroy SL, Clifford SC, Rutkowski S, Ellison DW, Malkin D, Taylor MD, Gajjar A, Pfister SM
Nat Rev Dis Primers 2019 Feb 14;5(1):11. doi: 10.1038/s41572-019-0063-6. PMID: 30765705
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article
Wells EM, Packer RJ
Continuum (Minneap Minn) 2015 Apr;21(2 Neuro-oncology):373-96. doi: 10.1212/01.CON.0000464176.96311.d1. PMID: 25837902

Therapy

Peyrl A, Chocholous M, Sabel M, Lassaletta A, Sterba J, Leblond P, Nysom K, Torsvik I, Chi SN, Perwein T, Jones N, Holm S, Nyman P, Mörse H, Öberg A, Weiler-Wichtl L, Leiss U, Haberler C, Schmook MT, Mayr L, Dieckmann K, Kool M, Gojo J, Azizi AA, André N, Kieran M, Slavc I
JAMA Oncol 2023 Dec 1;9(12):1688-1695. doi: 10.1001/jamaoncol.2023.4437. PMID: 37883081Free PMC Article
Gwynne WD, Suk Y, Custers S, Mikolajewicz N, Chan JK, Zador Z, Chafe SC, Zhai K, Escudero L, Zhang C, Zaslaver O, Chokshi C, Shaikh MV, Bakhshinyan D, Burns I, Chaudhry I, Nachmani O, Mobilio D, Maich WT, Mero P, Brown KR, Quaile AT, Venugopal C, Moffat J, Montenegro-Burke JR, Singh SK
Cancer Cell 2022 Dec 12;40(12):1488-1502.e7. Epub 2022 Nov 10 doi: 10.1016/j.ccell.2022.10.009. PMID: 36368321
Zhang M, Wong SW, Wright JN, Wagner MW, Toescu S, Han M, Tam LT, Zhou Q, Ahmadian SS, Shpanskaya K, Lummus S, Lai H, Eghbal A, Radmanesh A, Nemelka J, Harward S 2nd, Malinzak M, Laughlin S, Perreault S, Braun KRM, Lober RM, Cho YJ, Ertl-Wagner B, Ho CY, Mankad K, Vogel H, Cheshier SH, Jacques TS, Aquilina K, Fisher PG, Taylor M, Poussaint T, Vitanza NA, Grant GA, Pfister S, Thompson E, Jaju A, Ramaswamy V, Yeom KW
Radiology 2022 Aug;304(2):406-416. Epub 2022 Apr 19 doi: 10.1148/radiol.212137. PMID: 35438562Free PMC Article
Leary SES, Packer RJ, Li Y, Billups CA, Smith KS, Jaju A, Heier L, Burger P, Walsh K, Han Y, Embry L, Hadley J, Kumar R, Michalski J, Hwang E, Gajjar A, Pollack IF, Fouladi M, Northcott PA, Olson JM
JAMA Oncol 2021 Sep 1;7(9):1313-1321. doi: 10.1001/jamaoncol.2021.2224. PMID: 34292305Free PMC Article
Catsman-Berrevoets C, Patay Z
Handb Clin Neurol 2018;155:273-288. doi: 10.1016/B978-0-444-64189-2.00018-4. PMID: 29891065

Prognosis

Luo Z, Xia M, Shi W, Zhao C, Wang J, Xin D, Dong X, Xiong Y, Zhang F, Berry K, Ogurek S, Liu X, Rao R, Xing R, Wu LMN, Cui S, Xu L, Lin Y, Ma W, Tian S, Xie Q, Zhang L, Xin M, Wang X, Yue F, Zheng H, Liu Y, Stevenson CB, de Blank P, Perentesis JP, Gilbertson RJ, Li H, Ma J, Zhou W, Taylor MD, Lu QR
Nature 2022 Dec;612(7941):787-794. Epub 2022 Nov 30 doi: 10.1038/s41586-022-05487-2. PMID: 36450980
Vladoiu MC, El-Hamamy I, Donovan LK, Farooq H, Holgado BL, Sundaravadanam Y, Ramaswamy V, Hendrikse LD, Kumar S, Mack SC, Lee JJY, Fong V, Juraschka K, Przelicki D, Michealraj A, Skowron P, Luu B, Suzuki H, Morrissy AS, Cavalli FMG, Garzia L, Daniels C, Wu X, Qazi MA, Singh SK, Chan JA, Marra MA, Malkin D, Dirks P, Heisler L, Pugh T, Ng K, Notta F, Thompson EM, Kleinman CL, Joyner AL, Jabado N, Stein L, Taylor MD
Nature 2019 Aug;572(7767):67-73. Epub 2019 May 1 doi: 10.1038/s41586-019-1158-7. PMID: 31043743Free PMC Article
Northcott PA, Robinson GW, Kratz CP, Mabbott DJ, Pomeroy SL, Clifford SC, Rutkowski S, Ellison DW, Malkin D, Taylor MD, Gajjar A, Pfister SM
Nat Rev Dis Primers 2019 Feb 14;5(1):11. doi: 10.1038/s41572-019-0063-6. PMID: 30765705
Millard NE, De Braganca KC
J Child Neurol 2016 Oct;31(12):1341-53. Epub 2015 Sep 2 doi: 10.1177/0883073815600866. PMID: 26336203Free PMC Article
Wells EM, Packer RJ
Continuum (Minneap Minn) 2015 Apr;21(2 Neuro-oncology):373-96. doi: 10.1212/01.CON.0000464176.96311.d1. PMID: 25837902

Clinical prediction guides

Guo X, Chang M, Wang Y, Xing B, Ma W
Int J Biol Sci 2023;19(12):3762-3780. Epub 2023 Jul 24 doi: 10.7150/ijbs.85813. PMID: 37564196Free PMC Article
Luo Z, Xia M, Shi W, Zhao C, Wang J, Xin D, Dong X, Xiong Y, Zhang F, Berry K, Ogurek S, Liu X, Rao R, Xing R, Wu LMN, Cui S, Xu L, Lin Y, Ma W, Tian S, Xie Q, Zhang L, Xin M, Wang X, Yue F, Zheng H, Liu Y, Stevenson CB, de Blank P, Perentesis JP, Gilbertson RJ, Li H, Ma J, Zhou W, Taylor MD, Lu QR
Nature 2022 Dec;612(7941):787-794. Epub 2022 Nov 30 doi: 10.1038/s41586-022-05487-2. PMID: 36450980
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free PMC Article
Zhang M, Wong SW, Wright JN, Wagner MW, Toescu S, Han M, Tam LT, Zhou Q, Ahmadian SS, Shpanskaya K, Lummus S, Lai H, Eghbal A, Radmanesh A, Nemelka J, Harward S 2nd, Malinzak M, Laughlin S, Perreault S, Braun KRM, Lober RM, Cho YJ, Ertl-Wagner B, Ho CY, Mankad K, Vogel H, Cheshier SH, Jacques TS, Aquilina K, Fisher PG, Taylor M, Poussaint T, Vitanza NA, Grant GA, Pfister S, Thompson E, Jaju A, Ramaswamy V, Yeom KW
Radiology 2022 Aug;304(2):406-416. Epub 2022 Apr 19 doi: 10.1148/radiol.212137. PMID: 35438562Free PMC Article
Gajjar A, Robinson GW, Smith KS, Lin T, Merchant TE, Chintagumpala M, Mahajan A, Su J, Bouffet E, Bartels U, Schechter T, Hassall T, Robertson T, Nicholls W, Gururangan S, Schroeder K, Sullivan M, Wheeler G, Hansford JR, Kellie SJ, McCowage G, Cohn R, Fisher MJ, Krasin MJ, Stewart CF, Broniscer A, Buchhalter I, Tatevossian RG, Orr BA, Neale G, Klimo P Jr, Boop F, Srinivasan A, Pfister SM, Gilbertson RJ, Onar-Thomas A, Ellison DW, Northcott PA
J Clin Oncol 2021 Mar 1;39(7):822-835. Epub 2021 Jan 6 doi: 10.1200/JCO.20.01372. PMID: 33405951Free PMC Article

Recent systematic reviews

Nyalundja AD, Kanmounye US, Karekezi C, Laeke T, Thango N, Balogun JA
J Neurosurg Pediatr 2024 Jun 1;33(6):524-535. Epub 2024 Mar 15 doi: 10.3171/2024.1.PEDS23282. PMID: 38489811
Baqai MWS, Tariq R, Shah Z, Bajwa MH, Shamim MS
Childs Nerv Syst 2023 May;39(5):1159-1171. Epub 2023 Feb 24 doi: 10.1007/s00381-023-05886-4. PMID: 36826523
Shimazaki K, Kurokawa R, Franson A, Kurokawa M, Baba A, Bou-Maroun L, Kim J, Moritani T
J Neuroimaging 2023 May-Jun;33(3):359-367. Epub 2023 Feb 20 doi: 10.1111/jon.13095. PMID: 36806312
Alammar H, Nassani R, Alshehri MM, Aljohani AA, Alrfaei BM
Int J Mol Sci 2021 Dec 31;23(1) doi: 10.3390/ijms23010464. PMID: 35008889Free PMC Article
Wauters M, Uyttebroeck A, De Waele L, Sleurs C, Jacobs S
Cerebellum 2021 Jun;20(3):462-480. Epub 2021 Jan 8 doi: 10.1007/s12311-020-01225-4. PMID: 33417160

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