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Childhood central nervous system primitive neuroectodermal neoplasm

MedGen UID:
232347
Concept ID:
C1332957
Neoplastic Process
Synonyms: Central nervous system embryonal tumor, NOS; central nervous system primitive neuroectodermal neoplasm of childhood; Childhood Central Nervous System Embryonal Tumor, NOS; Childhood Central Nervous System Embryonal Tumor, Not Otherwise Specified; childhood central nervous system PNET; childhood central nervous system primitive neuroectodermal neoplasm; childhood central nervous system primitive neuroectodermal tumor; childhood central nervous system primitive neuroectodermal tumour; childhood central primitive neuroectodermal neoplasm; childhood central primitive neuroectodermal tumor; childhood central primitive neuroectodermal tumour; childhood CNS PNET; childhood CNS primitive neuroectodermal neoplasm; childhood CNS primitive neuroectodermal tumor; childhood CNS primitive neuroectodermal tumour; paediatric central nervous system primitive neuroectodermal neoplasm; paediatric central nervous system primitive neuroectodermal tumour; paediatric central primitive neuroectodermal neoplasm; paediatric central primitive neuroectodermal tumour; paediatric CNS PNET; paediatric CNS primitive neuroectodermal neoplasm; paediatric CNS primitive neuroectodermal tumour; pediatric central nervous system primitive neuroectodermal neoplasm; pediatric central nervous system primitive neuroectodermal tumor; pediatric central primitive neuroectodermal neoplasm; pediatric central primitive neuroectodermal tumor; pediatric CNS PNET; pediatric CNS primitive neuroectodermal neoplasm; pediatric CNS primitive neuroectodermal tumor
 
Monarch Initiative: MONDO:0002798

Definition

A central nervous system embryonal tumor, not otherwise specified that occurs in childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood central nervous system primitive neuroectodermal neoplasm

Professional guidelines

PubMed

Liu X, Ding C, Tan W, Zhang A
Pharmacol Ther 2020 Jun;210:107516. Epub 2020 Feb 24 doi: 10.1016/j.pharmthera.2020.107516. PMID: 32105673
Khanna G, Bushnell D, O'Dorisio MS
Oncologist 2008 Apr;13(4):382-9. doi: 10.1634/theoncologist.2007-0175. PMID: 18448552
Kortmann RD, Timmermann B, Becker G, Kühl J, Bamberg M
Klin Padiatr 1998 Jul-Aug;210(4):220-6. doi: 10.1055/s-2008-1043882. PMID: 9743956

Recent clinical studies

Etiology

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
Smith KS, Bihannic L, Gudenas BL, Haldipur P, Tao R, Gao Q, Li Y, Aldinger KA, Iskusnykh IY, Chizhikov VV, Scoggins M, Zhang S, Edwards A, Deng M, Glass IA, Overman LM, Millman J, Sjoboen AH, Hadley J, Golser J, Mankad K, Sheppard H, Onar-Thomas A, Gajjar A, Robinson GW, Hovestadt V, Orr BA, Patay Z, Millen KJ, Northcott PA
Nature 2022 Sep;609(7929):1012-1020. Epub 2022 Sep 21 doi: 10.1038/s41586-022-05208-9. PMID: 36131015Free PMC Article
Hovestadt V, Smith KS, Bihannic L, Filbin MG, Shaw ML, Baumgartner A, DeWitt JC, Groves A, Mayr L, Weisman HR, Richman AR, Shore ME, Goumnerova L, Rosencrance C, Carter RA, Phoenix TN, Hadley JL, Tong Y, Houston J, Ashmun RA, DeCuypere M, Sharma T, Flasch D, Silkov A, Ligon KL, Pomeroy SL, Rivera MN, Rozenblatt-Rosen O, Rusert JM, Wechsler-Reya RJ, Li XN, Peyrl A, Gojo J, Kirchhofer D, Lötsch D, Czech T, Dorfer C, Haberler C, Geyeregger R, Halfmann A, Gawad C, Easton J, Pfister SM, Regev A, Gajjar A, Orr BA, Slavc I, Robinson GW, Bernstein BE, Suvà ML, Northcott PA
Nature 2019 Aug;572(7767):74-79. Epub 2019 Jul 24 doi: 10.1038/s41586-019-1434-6. PMID: 31341285Free PMC Article
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

Diagnosis

Keck MK, Sill M, Wittmann A, Joshi P, Stichel D, Beck P, Okonechnikow K, Sievers P, Wefers AK, Roncaroli F, Avula S, McCabe MG, Hayden JT, Wesseling P, Øra I, Nistér M, Kranendonk MEG, Tops BBJ, Zapotocky M, Zamecnik J, Vasiljevic A, Fenouil T, Meyronet D, von Hoff K, Schüller U, Loiseau H, Figarella-Branger D, Kramm CM, Sturm D, Scheie D, Rauramaa T, Pesola J, Gojo J, Haberler C, Brandner S, Jacques T, Sexton Oates A, Saffery R, Koscielniak E, Baker SJ, Yip S, Snuderl M, Ud Din N, Samuel D, Schramm K, Blattner-Johnson M, Selt F, Ecker J, Milde T, von Deimling A, Korshunov A, Perry A, Pfister SM, Sahm F, Solomon DA, Jones DTW
Acta Neuropathol 2023 Jan;145(1):49-69. Epub 2022 Nov 27 doi: 10.1007/s00401-022-02516-2. PMID: 36437415Free PMC Article
Smith KS, Bihannic L, Gudenas BL, Haldipur P, Tao R, Gao Q, Li Y, Aldinger KA, Iskusnykh IY, Chizhikov VV, Scoggins M, Zhang S, Edwards A, Deng M, Glass IA, Overman LM, Millman J, Sjoboen AH, Hadley J, Golser J, Mankad K, Sheppard H, Onar-Thomas A, Gajjar A, Robinson GW, Hovestadt V, Orr BA, Patay Z, Millen KJ, Northcott PA
Nature 2022 Sep;609(7929):1012-1020. Epub 2022 Sep 21 doi: 10.1038/s41586-022-05208-9. PMID: 36131015Free 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
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Massimino M, Giangaspero F, Garrè ML, Gandola L, Poggi G, Biassoni V, Gatta G, Rutkowski S
Crit Rev Oncol Hematol 2011 Jul;79(1):65-83. Epub 2010 Dec 3 doi: 10.1016/j.critrevonc.2010.07.010. PMID: 21129995

Therapy

Chan GC, Chan CM
Biomolecules 2022 Feb 24;12(3) doi: 10.3390/biom12030358. PMID: 35327550Free PMC Article
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Liu X, Ding C, Tan W, Zhang A
Pharmacol Ther 2020 Jun;210:107516. Epub 2020 Feb 24 doi: 10.1016/j.pharmthera.2020.107516. PMID: 32105673
Coyle B, Kessler M, Sabnis DH, Kerr ID
Biochem Soc Trans 2015 Oct;43(5):1018-22. doi: 10.1042/BST20150137. PMID: 26517917
Johnsen JI, Kogner P, Albihn A, Henriksson MA
Apoptosis 2009 Apr;14(4):424-38. doi: 10.1007/s10495-009-0325-y. PMID: 19259824

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
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Bartlett F, Kortmann R, Saran F
Clin Oncol (R Coll Radiol) 2013 Jan;25(1):36-45. Epub 2012 Dec 11 doi: 10.1016/j.clon.2012.09.008. PMID: 23245832

Clinical prediction guides

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
Levy AS, Krailo M, Chi S, Villaluna D, Springer L, Williams-Hughes C, Fouladi M, Gajjar A
Pediatr Blood Cancer 2021 Aug;68(8):e29031. Epub 2021 Apr 12 doi: 10.1002/pbc.29031. PMID: 33844469Free PMC Article
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Venkatramani R, Pan H, Furman WL, Marron JM, Haduong J, Friedrich-Medina P, Mahajan A, Bavle A, Wu H, Chintagumpala M
Pediatr Blood Cancer 2016 Mar;63(3):465-70. Epub 2015 Oct 30 doi: 10.1002/pbc.25817. PMID: 26514449Free PMC Article
Sharp SE, Parisi MT, Gelfand MJ, Yanik GA, Shulkin BL
Q J Nucl Med Mol Imaging 2013 Mar;57(1):6-20. PMID: 23474631

Recent systematic reviews

Ajithkumar T, Avanzo M, Yorke E, Tsang DS, Milano MT, Olch AJ, Merchant TE, Dieckmann K, Mahajan A, Fuji H, Paulino AC, Timmermann B, Marks LB, Bentzen SM, Jackson A, Constine LS
Int J Radiat Oncol Biol Phys 2024 Jun 1;119(2):655-668. Epub 2024 Feb 1 doi: 10.1016/j.ijrobp.2023.12.043. PMID: 38300187
Paquette K, Coltin H, Boivin A, Amre D, Nuyt AM, Luu TM
PLoS One 2019;14(1):e0210366. Epub 2019 Jan 4 doi: 10.1371/journal.pone.0210366. PMID: 30608983Free PMC Article
Kreis C, Doessegger E, Lupatsch JE, Spycher BD
Eur J Epidemiol 2019 Jan;34(1):9-21. Epub 2018 Nov 16 doi: 10.1007/s10654-018-0456-y. PMID: 30446850Free PMC Article
Martin RM, Gunnell D, Owen CG, Smith GD
Int J Cancer 2005 Dec 20;117(6):1020-31. doi: 10.1002/ijc.21274. PMID: 15986434

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