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Childhood Brain Stem Mixed Glioma

MedGen UID:
854375
•Concept ID:
C3887516
•
Neoplastic Process

Definition

A mixed glioma that arises from the brain stem and occurs during childhood. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Brain Stem Mixed Glioma

Professional guidelines

PubMed

Contemporary Management of Pediatric Brainstem Tumors.
Chou SC, Chen YN, Huang HY, Kuo MF, Wong TT, Kuo SH, Yang SH
Adv Tech Stand Neurosurg 2024;49:231-254. doi: 10.1007/978-3-031-42398-7_11. PMID: 38700687
Immunotherapy approaches for the treatment of diffuse midline gliomas.
Bernstock JD, Hoffman SE, Kappel AD, Valdes PA, Essayed W, Klinger NV, Kang KD, Totsch SK, Olsen HE, Schlappi CW, Filipski K, Gessler FA, Baird L, Filbin MG, Hashizume R, Becher OJ, Friedman GK
Oncoimmunology 2022;11(1):2124058. Epub 2022 Sep 26 doi: 10.1080/2162402X.2022.2124058. PMID: 36185807Free PMC Article
Locoregional delivery of CAR T cells to the cerebrospinal fluid for treatment of metastatic medulloblastoma and ependymoma.
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

Recent clinical studies

Etiology

The landscape of tumor cell states and spatial organization in H3-K27M mutant diffuse midline glioma across age and location.
Liu I, Jiang L, Samuelsson ER, Marco Salas S, Beck A, Hack OA, Jeong D, Shaw ML, Englinger B, LaBelle J, Mire HM, Madlener S, Mayr L, Quezada MA, Trissal M, Panditharatna E, Ernst KJ, Vogelzang J, Gatesman TA, Halbert ME, Palova H, Pokorna P, Sterba J, Slaby O, Geyeregger R, Diaz A, Findlay IJ, Dun MD, Resnick A, Suvà ML, Jones DTW, Agnihotri S, Svedlund J, Koschmann C, Haberler C, Czech T, Slavc I, Cotter JA, Ligon KL, Alexandrescu S, Yung WKA, Arrillaga-Romany I, Gojo J, Monje M, Nilsson M, Filbin MG
Nat Genet 2022 Dec;54(12):1881-1894. Epub 2022 Dec 5 doi: 10.1038/s41588-022-01236-3. PMID: 36471067Free PMC Article
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
The whole-genome landscape of medulloblastoma subtypes.
Northcott PA, Buchhalter I, Morrissy AS, Hovestadt V, Weischenfeldt J, Ehrenberger T, Gröbner S, Segura-Wang M, Zichner T, Rudneva VA, Warnatz HJ, Sidiropoulos N, Phillips AH, Schumacher S, Kleinheinz K, Waszak SM, Erkek S, Jones DTW, Worst BC, Kool M, Zapatka M, Jäger N, Chavez L, Hutter B, Bieg M, Paramasivam N, Heinold M, Gu Z, Ishaque N, Jäger-Schmidt C, Imbusch CD, Jugold A, Hübschmann D, Risch T, Amstislavskiy V, Gonzalez FGR, Weber UD, Wolf S, Robinson GW, Zhou X, Wu G, Finkelstein D, Liu Y, Cavalli FMG, Luu B, Ramaswamy V, Wu X, Koster J, Ryzhova M, Cho YJ, Pomeroy SL, Herold-Mende C, Schuhmann M, Ebinger M, Liau LM, Mora J, McLendon RE, Jabado N, Kumabe T, Chuah E, Ma Y, Moore RA, Mungall AJ, Mungall KL, Thiessen N, Tse K, Wong T, Jones SJM, Witt O, Milde T, Von Deimling A, Capper D, Korshunov A, Yaspo ML, Kriwacki R, Gajjar A, Zhang J, Beroukhim R, Fraenkel E, Korbel JO, Brors B, Schlesner M, Eils R, Marra MA, Pfister SM, Taylor MD, Lichter P
Nature 2017 Jul 19;547(7663):311-317. doi: 10.1038/nature22973. PMID: 28726821Free PMC Article

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
Childhood Medulloblastoma: An Overview.
Suk Y, Gwynne WD, Burns I, Venugopal C, Singh SK
Methods Mol Biol 2022;2423:1-12. doi: 10.1007/978-1-0716-1952-0_1. PMID: 34978683
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free 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

Therapy

Intraventricular B7-H3 CAR T Cells for Diffuse Intrinsic Pontine Glioma: Preliminary First-in-Human Bioactivity and Safety.
Vitanza NA, Wilson AL, Huang W, Seidel K, Brown C, Gustafson JA, Yokoyama JK, Johnson AJ, Baxter BA, Koning RW, Reid AN, Meechan M, Biery MC, Myers C, Rawlings-Rhea SD, Albert CM, Browd SR, Hauptman JS, Lee A, Ojemann JG, Berens ME, Dun MD, Foster JB, Crotty EE, Leary SES, Cole BL, Perez FA, Wright JN, Orentas RJ, Chour T, Newell EW, Whiteaker JR, Zhao L, Paulovich AG, Pinto N, Gust J, Gardner RA, Jensen MC, Park JR
Cancer Discov 2023 Jan 9;13(1):114-131. doi: 10.1158/2159-8290.CD-22-0750. PMID: 36259971Free PMC Article
Immunotherapy approaches for the treatment of diffuse midline gliomas.
Bernstock JD, Hoffman SE, Kappel AD, Valdes PA, Essayed W, Klinger NV, Kang KD, Totsch SK, Olsen HE, Schlappi CW, Filipski K, Gessler FA, Baird L, Filbin MG, Hashizume R, Becher OJ, Friedman GK
Oncoimmunology 2022;11(1):2124058. Epub 2022 Sep 26 doi: 10.1080/2162402X.2022.2124058. PMID: 36185807Free PMC Article
A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study.
Fangusaro J, Onar-Thomas A, Poussaint TY, Wu S, Ligon AH, Lindeman N, Campagne O, Banerjee A, Gururangan S, Kilburn LB, Goldman S, Qaddoumi I, Baxter P, Vezina G, Bregman C, Patay Z, Jones JY, Stewart CF, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Neuro Oncol 2021 Oct 1;23(10):1777-1788. doi: 10.1093/neuonc/noab047. PMID: 33631016Free PMC Article
Locoregional delivery of CAR T cells to the cerebrospinal fluid for treatment of metastatic medulloblastoma and ependymoma.
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
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article

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
A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study.
Fangusaro J, Onar-Thomas A, Poussaint TY, Wu S, Ligon AH, Lindeman N, Campagne O, Banerjee A, Gururangan S, Kilburn LB, Goldman S, Qaddoumi I, Baxter P, Vezina G, Bregman C, Patay Z, Jones JY, Stewart CF, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Neuro Oncol 2021 Oct 1;23(10):1777-1788. doi: 10.1093/neuonc/noab047. PMID: 33631016Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free 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

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
Dormant SOX9-Positive Cells Facilitate MYC-Driven Recurrence of Medulloblastoma.
Borgenvik A, Holmberg KO, Bolin S, Zhao M, Savov V, Rosén G, Hutter S, Garancher A, Rahmanto AS, Bergström T, Olsen TK, Mainwaring OJ, Sattanino D, Verbaan AD, Rusert JM, Sundström A, Bravo MB, Dang Y, Wenz AS, Richardson S, Fotaki G, Hill RM, Dubuc AM, Kalushkova A, Remke M, Čančer M, Jernberg-Wiklund H, Giraud G, Chen X, Taylor MD, Sangfelt O, Clifford SC, Schüller U, Wechsler-Reya RJ, Weishaupt H, Swartling FJ
Cancer Res 2022 Dec 16;82(24):4586-4603. doi: 10.1158/0008-5472.CAN-22-2108. PMID: 36219398Free PMC Article
Selumetinib in paediatric patients with BRAF-aberrant or neurofibromatosis type 1-associated recurrent, refractory, or progressive low-grade glioma: a multicentre, phase 2 trial.
Fangusaro J, Onar-Thomas A, Young Poussaint T, Wu S, Ligon AH, Lindeman N, Banerjee A, Packer RJ, Kilburn LB, Goldman S, Pollack IF, Qaddoumi I, Jakacki RI, Fisher PG, Dhall G, Baxter P, Kreissman SG, Stewart CF, Jones DTW, Pfister SM, Vezina G, Stern JS, Panigrahy A, Patay Z, Tamrazi B, Jones JY, Haque SS, Enterline DS, Cha S, Fisher MJ, Doyle LA, Smith M, Dunkel IJ, Fouladi M
Lancet Oncol 2019 Jul;20(7):1011-1022. Epub 2019 May 28 doi: 10.1016/S1470-2045(19)30277-3. PMID: 31151904Free PMC Article
Spectrum and prevalence of genetic predisposition in medulloblastoma: a retrospective genetic study and prospective validation in a clinical trial cohort.
Waszak SM, Northcott PA, Buchhalter I, Robinson GW, Sutter C, Groebner S, Grund KB, Brugières L, Jones DTW, Pajtler KW, Morrissy AS, Kool M, Sturm D, Chavez L, Ernst A, Brabetz S, Hain M, Zichner T, Segura-Wang M, Weischenfeldt J, Rausch T, Mardin BR, Zhou X, Baciu C, Lawerenz C, Chan JA, Varlet P, Guerrini-Rousseau L, Fults DW, Grajkowska W, Hauser P, Jabado N, Ra YS, Zitterbart K, Shringarpure SS, De La Vega FM, Bustamante CD, Ng HK, Perry A, MacDonald TJ, Hernáiz Driever P, Bendel AE, Bowers DC, McCowage G, Chintagumpala MM, Cohn R, Hassall T, Fleischhack G, Eggen T, Wesenberg F, Feychting M, Lannering B, Schüz J, Johansen C, Andersen TV, Röösli M, Kuehni CE, Grotzer M, Kjaerheim K, Monoranu CM, Archer TC, Duke E, Pomeroy SL, Shelagh R, Frank S, Sumerauer D, Scheurlen W, Ryzhova MV, Milde T, Kratz CP, Samuel D, Zhang J, Solomon DA, Marra M, Eils R, Bartram CR, von Hoff K, Rutkowski S, Ramaswamy V, Gilbertson RJ, Korshunov A, Taylor MD, Lichter P, Malkin D, Gajjar A, Korbel JO, Pfister SM
Lancet Oncol 2018 Jun;19(6):785-798. Epub 2018 May 9 doi: 10.1016/S1470-2045(18)30242-0. PMID: 29753700Free PMC Article
Survival rates and prognostic predictors of high grade brain stem gliomas in childhood: a systematic review and meta-analysis.
Hassan H, Pinches A, Picton SV, Phillips RS
J Neurooncol 2017 Oct;135(1):13-20. Epub 2017 Jul 5 doi: 10.1007/s11060-017-2546-1. PMID: 28681244Free PMC Article

Recent systematic reviews

Medulloblastoma cancer stem cells: molecular signatures and therapeutic targets.
Bahmad HF, Poppiti RJ
J Clin Pathol 2020 May;73(5):243-249. Epub 2020 Feb 7 doi: 10.1136/jclinpath-2019-206246. PMID: 32034059
Survival rates and prognostic predictors of high grade brain stem gliomas in childhood: a systematic review and meta-analysis.
Hassan H, Pinches A, Picton SV, Phillips RS
J Neurooncol 2017 Oct;135(1):13-20. Epub 2017 Jul 5 doi: 10.1007/s11060-017-2546-1. PMID: 28681244Free PMC Article

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