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Adult central nervous system primitive neuroectodermal neoplasm

MedGen UID:
231039
Concept ID:
C1332196
Neoplastic Process
Synonyms: Adult Central Nervous System Embryonal Tumor, NOS; Adult Central Nervous System Embryonal Tumor, Not Otherwise Specified; adult central nervous system primitive neuroectodermal neoplasm; adult central nervous system primitive neuroectodermal tumor; adult central nervous system primitive neuroectodermal tumour; adult central primitive neuroectodermal neoplasm; adult central primitive neuroectodermal tumor; adult central primitive neuroectodermal tumour; adult CNS PNET; adult CNS primitive neuroectodermal neoplasm; adult CNS primitive neuroectodermal tumor; adult CNS primitive neuroectodermal tumour; central nervous system primitive neuroectodermal neoplasm of adults
 
Monarch Initiative: MONDO:0002795

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

Lu X, Zhang X, Deng X, Yang Z, Shen X, Sheng H, Yin B, Zhang N, Lin J
World Neurosurg 2020 Aug;140:e61-e72. Epub 2020 Apr 25 doi: 10.1016/j.wneu.2020.04.145. PMID: 32344130
Karsy M, Guan J, Sivakumar W, Neil JA, Schmidt MH, Mahan MA
Neurosurg Focus 2015 Aug;39(2):E3. doi: 10.3171/2015.5.FOCUS15143. PMID: 26235020
Kostaras X, Easaw JC
J Neurooncol 2013 Oct;115(1):1-8. Epub 2013 Jul 23 doi: 10.1007/s11060-013-1206-3. PMID: 23877361

Recent clinical studies

Etiology

Hendrikse LD, Haldipur P, Saulnier O, Millman J, Sjoboen AH, Erickson AW, Ong W, Gordon V, Coudière-Morrison L, Mercier AL, Shokouhian M, Suárez RA, Ly M, Borlase S, Scott DS, Vladoiu MC, Farooq H, Sirbu O, Nakashima T, Nambu S, Funakoshi Y, Bahcheli A, Diaz-Mejia JJ, Golser J, Bach K, Phuong-Bao T, Skowron P, Wang EY, Kumar SA, Balin P, Visvanathan A, Lee JJY, Ayoub R, Chen X, Chen X, Mungall KL, Luu B, Bérubé P, Wang YC, Pfister SM, Kim SK, Delattre O, Bourdeaut F, Doz F, Masliah-Planchon J, Grajkowska WA, Loukides J, Dirks P, Fèvre-Montange M, Jouvet A, French PJ, Kros JM, Zitterbart K, Bailey SD, Eberhart CG, Rao AAN, Giannini C, Olson JM, Garami M, Hauser P, Phillips JJ, Ra YS, de Torres C, Mora J, Li KKW, Ng HK, Poon WS, Pollack IF, López-Aguilar E, Gillespie GY, Van Meter TE, Shofuda T, Vibhakar R, Thompson RC, Cooper MK, Rubin JB, Kumabe T, Jung S, Lach B, Iolascon A, Ferrucci V, de Antonellis P, Zollo M, Cinalli G, Robinson S, Stearns DS, Van Meir EG, Porrati P, Finocchiaro G, Massimino M, Carlotti CG, Faria CC, Roussel MF, Boop F, Chan JA, Aldinger KA, Razavi F, Silvestri E, McLendon RE, Thompson EM, Ansari M, Garre ML, Chico F, Eguía P, Pérezpeña M, Morrissy AS, Cavalli FMG, Wu X, Daniels C, Rich JN, Jones SJM, Moore RA, Marra MA, Huang X, Reimand J, Sorensen PH, Wechsler-Reya RJ, Weiss WA, Pugh TJ, Garzia L, Kleinman CL, Stein LD, Jabado N, Malkin D, Ayrault O, Golden JA, Ellison DW, Doble B, Ramaswamy V, Werbowetski-Ogilvie TE, Suzuki H, Millen KJ, Taylor MD
Nature 2022 Sep;609(7929):1021-1028. Epub 2022 Sep 21 doi: 10.1038/s41586-022-05215-w. PMID: 36131014Free PMC Article
Skowron P, Farooq H, Cavalli FMG, Morrissy AS, Ly M, Hendrikse LD, Wang EY, Djambazian H, Zhu H, Mungall KL, Trinh QM, Zheng T, Dai S, Stucklin ASG, Vladoiu MC, Fong V, Holgado BL, Nor C, Wu X, Abd-Rabbo D, Bérubé P, Wang YC, Luu B, Suarez RA, Rastan A, Gillmor AH, Lee JJY, Zhang XY, Daniels C, Dirks P, Malkin D, Bouffet E, Tabori U, Loukides J, Doz FP, Bourdeaut F, Delattre OO, Masliah-Planchon J, Ayrault O, Kim SK, Meyronet D, Grajkowska WA, Carlotti CG, de Torres C, Mora J, Eberhart CG, Van Meir EG, Kumabe T, French PJ, Kros JM, Jabado N, Lach B, Pollack IF, Hamilton RL, Rao AAN, Giannini C, Olson JM, Bognár L, Klekner A, Zitterbart K, Phillips JJ, Thompson RC, Cooper MK, Rubin JB, Liau LM, Garami M, Hauser P, Li KKW, Ng HK, Poon WS, Yancey Gillespie G, Chan JA, Jung S, McLendon RE, Thompson EM, Zagzag D, Vibhakar R, Ra YS, Garre ML, Schüller U, Shofuda T, Faria CC, López-Aguilar E, Zadeh G, Hui CC, Ramaswamy V, Bailey SD, Jones SJ, Mungall AJ, Moore RA, Calarco JA, Stein LD, Bader GD, Reimand J, Ragoussis J, Weiss WA, Marra MA, Suzuki H, Taylor MD
Nat Commun 2021 Mar 19;12(1):1749. doi: 10.1038/s41467-021-21883-0. PMID: 33741928Free 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
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
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

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
Coltin H, Sundaresan L, Smith KS, Skowron P, Massimi L, Eberhart CG, Schreck KC, Gupta N, Weiss WA, Tirapelli D, Carlotti C, Li KKW, Ryzhova M, Golanov A, Zheludkova O, Absalyamova O, Okonechnikov K, Stichel D, von Deimling A, Giannini C, Raskin S, Van Meir EG, Chan JA, Fults D, Chambless LB, Kim SK, Vasiljevic A, Faure-Conter C, Vibhakar R, Jung S, Leary S, Mora J, McLendon RE, Pollack IF, Hauser P, Grajkowska WA, Rubin JB, van Veelen MC, French PJ, Kros JM, Liau LM, Pfister SM, Kool M, Kijima N, Taylor MD, Packer RJ, Northcott PA, Korshunov A, Ramaswamy V
Acta Neuropathol 2021 Nov;142(5):859-871. Epub 2021 Aug 18 doi: 10.1007/s00401-021-02358-4. PMID: 34409497Free PMC Article
Guerreiro Stucklin AS, Grotzer MA
Handb Clin Neurol 2018;155:289-299. doi: 10.1016/B978-0-444-64189-2.00019-6. PMID: 29891066
Sturm D, Orr BA, Toprak UH, Hovestadt V, Jones DTW, Capper D, Sill M, Buchhalter I, Northcott PA, Leis I, Ryzhova M, Koelsche C, Pfaff E, Allen SJ, Balasubramanian G, Worst BC, Pajtler KW, Brabetz S, Johann PD, Sahm F, Reimand J, Mackay A, Carvalho DM, Remke M, Phillips JJ, Perry A, Cowdrey C, Drissi R, Fouladi M, Giangaspero F, Łastowska M, Grajkowska W, Scheurlen W, Pietsch T, Hagel C, Gojo J, Lötsch D, Berger W, Slavc I, Haberler C, Jouvet A, Holm S, Hofer S, Prinz M, Keohane C, Fried I, Mawrin C, Scheie D, Mobley BC, Schniederjan MJ, Santi M, Buccoliero AM, Dahiya S, Kramm CM, von Bueren AO, von Hoff K, Rutkowski S, Herold-Mende C, Frühwald MC, Milde T, Hasselblatt M, Wesseling P, Rößler J, Schüller U, Ebinger M, Schittenhelm J, Frank S, Grobholz R, Vajtai I, Hans V, Schneppenheim R, Zitterbart K, Collins VP, Aronica E, Varlet P, Puget S, Dufour C, Grill J, Figarella-Branger D, Wolter M, Schuhmann MU, Shalaby T, Grotzer M, van Meter T, Monoranu CM, Felsberg J, Reifenberger G, Snuderl M, Forrester LA, Koster J, Versteeg R, Volckmann R, van Sluis P, Wolf S, Mikkelsen T, Gajjar A, Aldape K, Moore AS, Taylor MD, Jones C, Jabado N, Karajannis MA, Eils R, Schlesner M, Lichter P, von Deimling A, Pfister SM, Ellison DW, Korshunov A, Kool M
Cell 2016 Feb 25;164(5):1060-1072. doi: 10.1016/j.cell.2016.01.015. PMID: 26919435Free PMC Article
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

Liu IC, Holtzman AL, Rotondo RL, Indelicato DJ, Gururangan S, Cavaliere R, Carter B, Morris CG, Tavanaiepour D, Rutenberg MS
J Neurooncol 2021 Jul;153(3):467-476. Epub 2021 Jun 8 doi: 10.1007/s11060-021-03783-x. PMID: 34105033
Berlanga P, Pasqualini C, Pötschger U, Sangüesa C, Castellani MR, Cañete A, Luksch R, Elliot M, Schreier G, Kropf M, Morgenstern D, Papadakis V, Ash S, Ruud E, Brock P, Wieczorek A, Kogner P, Trahair T, Ambros P, Boterberg T, Castel V, Valteau-Couanet D, Ladenstein R
Eur J Cancer 2021 Feb;144:1-8. Epub 2020 Dec 11 doi: 10.1016/j.ejca.2020.10.020. PMID: 33316634
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
Guerreiro Stucklin AS, Grotzer MA
Handb Clin Neurol 2018;155:289-299. doi: 10.1016/B978-0-444-64189-2.00019-6. PMID: 29891066
Carceller F, Bautista F, Jiménez I, Hladun-Álvaro R, Giraud C, Bergamaschi L, Dandapani M, Aerts I, Doz F, Frappaz D, Casanova M, Morland B, Hargrave DR, Vassal G, Pearson ADJ, Geoerger B, Moreno L, Marshall LV
J Neurooncol 2018 Mar;137(1):83-92. Epub 2017 Dec 13 doi: 10.1007/s11060-017-2698-z. PMID: 29236237

Prognosis

Coltin H, Sundaresan L, Smith KS, Skowron P, Massimi L, Eberhart CG, Schreck KC, Gupta N, Weiss WA, Tirapelli D, Carlotti C, Li KKW, Ryzhova M, Golanov A, Zheludkova O, Absalyamova O, Okonechnikov K, Stichel D, von Deimling A, Giannini C, Raskin S, Van Meir EG, Chan JA, Fults D, Chambless LB, Kim SK, Vasiljevic A, Faure-Conter C, Vibhakar R, Jung S, Leary S, Mora J, McLendon RE, Pollack IF, Hauser P, Grajkowska WA, Rubin JB, van Veelen MC, French PJ, Kros JM, Liau LM, Pfister SM, Kool M, Kijima N, Taylor MD, Packer RJ, Northcott PA, Korshunov A, Ramaswamy V
Acta Neuropathol 2021 Nov;142(5):859-871. Epub 2021 Aug 18 doi: 10.1007/s00401-021-02358-4. PMID: 34409497Free PMC Article
Spino M, Snuderl M
Adv Anat Pathol 2020 Jan;27(1):44-50. doi: 10.1097/PAP.0000000000000254. PMID: 31714292
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
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
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

Clinical prediction guides

Fallah J, Brave MH, Weinstock C, Mehta GU, Bradford D, Gittleman H, Bloomquist EW, Charlab R, Hamed SS, Miller CP, Dorff SE, Chambers WA, Mixter BD, Dinin J, Pierce WF, Ricks TK, Tang S, Donoghue M, Pazdur R, Amiri-Kordestani L, Ibrahim A, Beaver JA
Clin Cancer Res 2022 Nov 14;28(22):4843-4848. doi: 10.1158/1078-0432.CCR-22-1054. PMID: 35727604Free PMC Article
Lyu L, Zou L, Jiang Y, Ding H, Chen C, Ye F, Hu Y, Yin S, Zhou P, Jiang S
J Clin Neurosci 2020 Nov;81:83-89. Epub 2020 Sep 29 doi: 10.1016/j.jocn.2020.09.009. PMID: 33222976
Spino M, Snuderl M
Adv Anat Pathol 2020 Jan;27(1):44-50. doi: 10.1097/PAP.0000000000000254. PMID: 31714292
Wegmann S, Eftekharzadeh B, Tepper K, Zoltowska KM, Bennett RE, Dujardin S, Laskowski PR, MacKenzie D, Kamath T, Commins C, Vanderburg C, Roe AD, Fan Z, Molliex AM, Hernandez-Vega A, Muller D, Hyman AA, Mandelkow E, Taylor JP, Hyman BT
EMBO J 2018 Apr 3;37(7) Epub 2018 Feb 22 doi: 10.15252/embj.201798049. PMID: 29472250Free PMC Article
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

Recent systematic reviews

Mozaffari K, Pradhan A, Yang I, Patel K, Vivas AC
J Neurol Sci 2022 Nov 15;442:120406. Epub 2022 Sep 1 doi: 10.1016/j.jns.2022.120406. PMID: 36081302
Sarmiento BE, Callegari S, Ghotme KA, Akle V
Cells 2022 Apr 2;11(7) doi: 10.3390/cells11071204. PMID: 35406768Free PMC Article
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
Kostaras X, Easaw JC
J Neurooncol 2013 Oct;115(1):1-8. Epub 2013 Jul 23 doi: 10.1007/s11060-013-1206-3. PMID: 23877361
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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