U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Medulloblastoma: Molecular understanding, treatment evolution, and new developments.
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
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
Diagnosis and management of brain and spinal cord tumors in the neonate.
Hwang SW, Su JM, Jea A
Semin Fetal Neonatal Med 2012 Aug;17(4):202-206. Epub 2012 Mar 30 doi: 10.1016/j.siny.2012.03.001. PMID: 22465555

Recent clinical studies

Etiology

Human fetal cerebellar cell atlas informs medulloblastoma origin and oncogenesis.
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
Anti-GD2 Directed Immunotherapy for High-Risk and Metastatic Neuroblastoma.
Chan GC, Chan CM
Biomolecules 2022 Feb 24;12(3) doi: 10.3390/biom12030358. PMID: 35327550Free PMC Article
Resolving medulloblastoma cellular architecture by single-cell genomics.
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
Childhood cerebellar tumours mirror conserved fetal transcriptional programs.
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
Medulloblastoma.
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

Unified rhombic lip origins of group 3 and group 4 medulloblastoma.
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
Medulloblastoma.
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
Childhood Medulloblastoma Revisited.
Cassia GSE, Alves CAPF, Taranath A, López NS, Oztekin O, Gonçalves FG, Patay Z
Top Magn Reson Imaging 2018 Dec;27(6):479-502. doi: 10.1097/RMR.0000000000000184. PMID: 30516696
Biological and Genetic Features of Neuroblastoma and Their Clinical Importance.
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Childhood medulloblastoma.
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

Paediatric Strategy Forum for medicinal product development of DNA damage response pathway inhibitors in children and adolescents with cancer: ACCELERATE in collaboration with the European Medicines Agency with participation of the Food and Drug Administration.
Pearson ADJ, Federico S, Gatz SA, Ortiz M, Lesa G, Scobie N, Gounaris I, Weiner SL, Weigel B, Unger TJ, Stewart E, Smith M, Slotkin EK, Reaman G, Pappo A, Nysom K, Norga K, McDonough J, Marshall LV, Ludwinski D, Ligas F, Karres D, Kool M, Horner TJ, Henssen A, Heenen D, Hawkins DS, Gore L, Bender JG, Galluzzo S, Fox E, de Rojas T, Davies BR, Chakrabarti J, Carmichael J, Bradford D, Blanc P, Bernardi R, Benchetrit S, Akindele K, Vassal G
Eur J Cancer 2023 Sep;190:112950. Epub 2023 Jun 21 doi: 10.1016/j.ejca.2023.112950. PMID: 37441939
Anti-GD2 Directed Immunotherapy for High-Risk and Metastatic Neuroblastoma.
Chan GC, Chan CM
Biomolecules 2022 Feb 24;12(3) doi: 10.3390/biom12030358. PMID: 35327550Free PMC Article
Temozolomide with irinotecan versus temozolomide, irinotecan plus bevacizumab for recurrent medulloblastoma of childhood: Report of a COG randomized Phase II screening trial.
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
Medulloblastoma: Molecular understanding, treatment evolution, and new developments.
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
Medulloblastoma and central nervous system germ cell tumors in adults: is pediatric experience applicable?
Mascarin M, Coassin E, Franceschi E, Gandola L, Carrabba G, Brandes AA, Massimino M
Childs Nerv Syst 2019 Dec;35(12):2279-2287. Epub 2019 Aug 14 doi: 10.1007/s00381-019-04340-8. PMID: 31414168

Prognosis

Human fetal cerebellar cell atlas informs medulloblastoma origin and oncogenesis.
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
Childhood cerebellar tumours mirror conserved fetal transcriptional programs.
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
Medulloblastoma.
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
Biological and Genetic Features of Neuroblastoma and Their Clinical Importance.
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Medulloblastoma.
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

Human fetal cerebellar cell atlas informs medulloblastoma origin and oncogenesis.
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
Development of GPC2-directed chimeric antigen receptors using mRNA for pediatric brain tumors.
Foster JB, Griffin C, Rokita JL, Stern A, Brimley C, Rathi K, Lane MV, Buongervino SN, Smith T, Madsen PJ, Martinez D, Delaidelli A, Sorensen PH, Wechsler-Reya RJ, Karikó K, Storm PB, Barrett DM, Resnick AC, Maris JM, Bosse KR
J Immunother Cancer 2022 Sep;10(9) doi: 10.1136/jitc-2021-004450. PMID: 36167467Free PMC Article
Biological and Genetic Features of Neuroblastoma and Their Clinical Importance.
Aygun N
Curr Pediatr Rev 2018;14(2):73-90. doi: 10.2174/1573396314666180129101627. PMID: 29380702
Childhood medulloblastoma.
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
Childhood brain tumors.
Sklar CA
J Pediatr Endocrinol Metab 2002 May;15 Suppl 2:669-73. doi: 10.1515/jpem.2002.15.s2.669. PMID: 12092679

Recent systematic reviews

Cancer risk in children and young adults born preterm: A systematic review and meta-analysis.
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
Space-time clustering of childhood cancers: a systematic review and pooled analysis.
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
Breast-feeding and childhood cancer: A systematic review with metaanalysis.
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...