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Medulloblastoma WNT activated

MedGen UID:
1392356
•Concept ID:
C4331965
•
Neoplastic Process
Synonyms: medulloblastoma WNT activated; Medulloblastoma, WNT-Activated
 
Monarch Initiative: MONDO:0850196

Definition

A molecular subtype of medulloblastoma associated with activation of the WNT pathway. TP53 mutations may be present or absent. WNT pathway activation in medulloblastomas is associated with good outcome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedulloblastoma WNT activated

Professional guidelines

PubMed

A Pilot Study Omitting Radiation in the Treatment of Children with Newly Diagnosed Wnt-Activated Medulloblastoma.
Cohen KJ, Munjapara V, Aguilera D, Castellino RC, Stapleton SL, Landi D, Ashley DM, Rodriguez FJ, Hawkins C, Yang E, London W, Chi S, Bandopadhayay P
Clin Cancer Res 2023 Dec 15;29(24):5031-5037. doi: 10.1158/1078-0432.CCR-23-0348. PMID: 37498309
Genetic Markers as Predictors for Response to Treatment and Possible Therapeutic Targets in Medulloblastoma.
Pérez-Pineda PL, Ortiz-Butrón R, Pérez-De Marcos JC, Hernández-Regino LM, Zapata-Tarrés MM, Torres-Espíndola LM
CNS Neurol Disord Drug Targets 2023;22(5):634-642. doi: 10.2174/1871527321666220509141030. PMID: 35579144
Pattern of Relapse and Treatment Response in WNT-Activated Medulloblastoma.
Nobre L, Zapotocky M, Khan S, Fukuoka K, Fonseca A, McKeown T, Sumerauer D, Vicha A, Grajkowska WA, Trubicka J, Li KKW, Ng HK, Massimi L, Lee JY, Kim SK, Zelcer S, Vasiljevic A, Faure-Conter C, Hauser P, Lach B, van Veelen-Vincent ML, French PJ, Van Meir EG, Weiss WA, Gupta N, Pollack IF, Hamilton RL, Nageswara Rao AA, Giannini C, Rubin JB, Moore AS, Chambless LB, Vibhakar R, Ra YS, Massimino M, McLendon RE, Wheeler H, Zollo M, Ferruci V, Kumabe T, Faria CC, Sterba J, Jung S, López-Aguilar E, Mora J, Carlotti CG, Olson JM, Leary S, Cain J, Krskova L, Zamecnik J, Hawkins CE, Tabori U, Huang A, Bartels U, Northcott PA, Taylor MD, Yip S, Hansford JR, Bouffet E, Ramaswamy V
Cell Rep Med 2020 Jun 23;1(3) doi: 10.1016/j.xcrm.2020.100038. PMID: 32743560Free PMC Article

Recent clinical studies

Etiology

Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group.
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
Molecular pathology of tumors of the central nervous system.
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
Childhood brain tumors: current management, biological insights, and future directions.
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article
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
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

Medulloblastoma: WHO 2021 and Beyond.
Cotter JA, Hawkins C
Pediatr Dev Pathol 2022 Jan-Feb;25(1):23-33. doi: 10.1177/10935266211018931. PMID: 35168417
Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group.
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
Molecular pathology of tumors of the central nervous system.
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
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
The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.
Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK, Ohgaki H, Wiestler OD, Kleihues P, Ellison DW
Acta Neuropathol 2016 Jun;131(6):803-20. Epub 2016 May 9 doi: 10.1007/s00401-016-1545-1. PMID: 27157931

Therapy

Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group.
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
Molecular pathology of tumors of the central nervous system.
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
Genomic testing, tumor microenvironment and targeted therapy of Hedgehog-related human cancers.
Katoh M
Clin Sci (Lond) 2019 Apr 30;133(8):953-970. Epub 2019 Apr 29 doi: 10.1042/CS20180845. PMID: 31036756
Childhood brain tumors: current management, biological insights, and future directions.
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article
Medulloblastoma stem cells.
Fan X, Eberhart CG
J Clin Oncol 2008 Jun 10;26(17):2821-7. doi: 10.1200/JCO.2007.15.2264. PMID: 18539960Free PMC Article

Prognosis

GD2-Targeting CAR T-cell Therapy for Patients with GD2+ Medulloblastoma.
Ciccone R, Quintarelli C, Camera A, Pezzella M, Caruso S, Manni S, Ottaviani A, Guercio M, Del Bufalo F, Quadraccia MC, Orlando D, Di Cecca S, Sinibaldi M, Aurigemma M, Iaffaldano L, Sarcinelli A, D'Amore ML, Ceccarelli M, Nazio F, Marabitti V, Giorda E, Pezzullo M, De Stefanis C, Carai A, Rossi S, Alaggio R, Del Baldo G, Becilli M, Mastronuzzi A, De Angelis B, Locatelli F
Clin Cancer Res 2024 Jun 3;30(11):2545-2557. doi: 10.1158/1078-0432.CCR-23-1880. PMID: 38551501Free PMC Article
Epigenetic upregulation of Schlafen11 renders 
WNT- and SHH-activated medulloblastomas sensitive to cisplatin.
Nakata S, Murai J, Okada M, Takahashi H, Findlay TH, Malebranche K, Parthasarathy A, Miyashita S, Gabdulkhaev R, Benkimoun I, Druillennec S, Chabi S, Hawkins E, Miyahara H, Tateishi K, Yamashita S, Yamada S, Saito T, On J, Watanabe J, Tsukamoto Y, Yoshimura J, Oishi M, Nakano T, Imamura M, Imai C, Yamamoto T, Takeshima H, Sasaki AT, Rodriguez FJ, Nobusawa S, Varlet P, Pouponnot C, Osuka S, Pommier Y, Kakita A, Fujii Y, Raabe EH, Eberhart CG, Natsumeda M
Neuro Oncol 2023 May 4;25(5):899-912. doi: 10.1093/neuonc/noac243. PMID: 36273330Free PMC Article
Molecular pathology of tumors of the central nervous system.
Kristensen BW, Priesterbach-Ackley LP, Petersen JK, Wesseling P
Ann Oncol 2019 Aug 1;30(8):1265-1278. doi: 10.1093/annonc/mdz164. PMID: 31124566Free PMC Article
Childhood brain tumors: current management, biological insights, and future directions.
Pollack IF, Agnihotri S, Broniscer A
J Neurosurg Pediatr 2019 Mar 1;23(3):261-273. doi: 10.3171/2018.10.PEDS18377. PMID: 30835699Free PMC Article
Medulloblastoma, WNT-activated/SHH-activated: clinical impact of molecular analysis and histogenetic evaluation.
Cambruzzi E
Childs Nerv Syst 2018 May;34(5):809-815. Epub 2018 Mar 26 doi: 10.1007/s00381-018-3765-2. PMID: 29582169

Clinical prediction guides

Single-Cell Chromatin Accessibility Analysis Reveals Subgroup-Specific TF-NTR Regulatory Circuits in Medulloblastoma.
Gao X, Zhuang Q, Li Y, Li G, Huang Z, Chen S, Sun S, Yang H, Jiang L, Mao Y
Adv Sci (Weinh) 2024 Aug;11(30):e2309554. Epub 2024 Jun 17 doi: 10.1002/advs.202309554. PMID: 38884167Free PMC Article
Gene-Specific DNA Methylation Profiles in Pediatric Medulloblastomas.
Kanit N, Uysal Yoca O, Ince D, Olgun N, Ozer E
Pediatr Dev Pathol 2022 Mar-Apr;25(2):82-90. Epub 2021 Sep 23 doi: 10.1177/10935266211036680. PMID: 34554028
Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group.
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
Immunohistochemical and nanoString-Based Subgrouping of Clinical Medulloblastoma Samples.
D'Arcy CE, Nobre LF, Arnaldo A, Ramaswamy V, Taylor MD, Naz-Hazrati L, Hawkins CE
J Neuropathol Exp Neurol 2020 Apr 1;79(4):437-447. doi: 10.1093/jnen/nlaa005. PMID: 32053195
Are molecular subgroups of medulloblastomas really prognostic?
Frappaz D, Faure-Conter C, Meyronet D, Levard-Bonneville A, Beuriat PA, Sunyach MP, Barritault M
Curr Opin Neurol 2018 Dec;31(6):747-751. doi: 10.1097/WCO.0000000000000626. PMID: 30300240

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