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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

Failure of human rhombic lip differentiation underlies medulloblastoma formation.
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
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
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
The rationale for targeted therapies in medulloblastoma.
MacDonald TJ, Aguilera D, Castellino RC
Neuro Oncol 2014 Jan;16(1):9-20. Epub 2013 Dec 4 doi: 10.1093/neuonc/not147. PMID: 24305711Free PMC Article
Medulloblastoma: signalling a change in treatment.
Gilbertson RJ
Lancet Oncol 2004 Apr;5(4):209-18. doi: 10.1016/S1470-2045(04)01424-X. PMID: 15050952

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
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
The rationale for targeted therapies in medulloblastoma.
MacDonald TJ, Aguilera D, Castellino RC
Neuro Oncol 2014 Jan;16(1):9-20. Epub 2013 Dec 4 doi: 10.1093/neuonc/not147. PMID: 24305711Free 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

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
G9a/EHMT2 is a Potential Prognostic Biomarker and Molecular Target in SHH Medulloblastoma.
Souza BK, Freire NH, Jaeger M, de Farias CB, Brunetto AL, Brunetto AT, Roesler R
Neuromolecular Med 2022 Dec;24(4):392-398. Epub 2022 Feb 3 doi: 10.1007/s12017-022-08702-5. PMID: 35113321
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: signalling a change in treatment.
Gilbertson RJ
Lancet Oncol 2004 Apr;5(4):209-18. doi: 10.1016/S1470-2045(04)01424-X. PMID: 15050952

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
G9a/EHMT2 is a Potential Prognostic Biomarker and Molecular Target in SHH Medulloblastoma.
Souza BK, Freire NH, Jaeger M, de Farias CB, Brunetto AL, Brunetto AT, Roesler R
Neuromolecular Med 2022 Dec;24(4):392-398. Epub 2022 Feb 3 doi: 10.1007/s12017-022-08702-5. PMID: 35113321
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
A CK1 FRET biosensor reveals that DDX3X is an essential activator of CK1ε.
Dolde C, Bischof J, Grüter S, Montada A, Halekotte J, Peifer C, Kalbacher H, Baumann U, Knippschild U, Suter B
J Cell Sci 2018 Jan 4;131(1) doi: 10.1242/jcs.207316. PMID: 29222110Free PMC Article
Presenilin couples the paired phosphorylation of beta-catenin independent of axin: implications for beta-catenin activation in tumorigenesis.
Kang DE, Soriano S, Xia X, Eberhart CG, De Strooper B, Zheng H, Koo EH
Cell 2002 Sep 20;110(6):751-62. doi: 10.1016/s0092-8674(02)00970-4. PMID: 12297048

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