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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

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
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
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

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
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
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
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
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

Cotter JA, Hawkins C
Pediatr Dev Pathol 2022 Jan-Feb;25(1):23-33. doi: 10.1177/10935266211018931. PMID: 35168417
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
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
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
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

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
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
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
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
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

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
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
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
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
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

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
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
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Narayan V, Sugur H, Jaiswal J, Arvinda HR, Arivazhagan A, Somanna S, Santosh V
Pediatr Neurosurg 2019;54(5):329-340. Epub 2019 Sep 3 doi: 10.1159/000501913. PMID: 31480064
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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