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Recurrent Medulloblastoma, WNT-Activated

MedGen UID:
1793138
•Concept ID:
C5556697
•
Neoplastic Process

Definition

The reemergence of medulloblastoma, WNT-activated after a period of remission. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRecurrent Medulloblastoma, 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
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
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article

Recent clinical studies

Etiology

Genetic alterations of TP53 and OTX2 indicate increased risk of relapse in WNT medulloblastomas.
Goschzik T, Mynarek M, Doerner E, Schenk A, Spier I, Warmuth-Metz M, Bison B, Obrecht D, Struve N, Kortmann RD, Schmid M, Aretz S, Rutkowski S, Pietsch T
Acta Neuropathol 2022 Dec;144(6):1143-1156. Epub 2022 Oct 1 doi: 10.1007/s00401-022-02505-5. PMID: 36181537Free PMC Article
Exclusive Hyperfractionated Radiation Therapy and Reduced Boost Volume for Standard-Risk Medulloblastoma: Pooled Analysis of the 2 French Multicentric Studies MSFOP98 and MSFOP 2007 and Correlation With Molecular Subgroups.
Carrie C, Kieffer V, Figarella-Branger D, Masliah-Planchon J, Bolle S, Bernier V, Laprie A, Supiot S, Leseur J, Habrand JL, Alapetite C, Kerr C, Dufour C, Claude L, Chapet S, Huchet A, Bondiau PY, Escande A, Truc G, Nguyen TD, Pasteuris C, Vigneron C, Muracciole X, Bourdeaut F, Appay R, Dubray B, Colin C, Ferlay C, Dussart S, Chabaud S, Padovani L; French Group of Pediatric Radiotherapy (GFRP); French Society of Pediatric Cancers (SFCE)
Int J Radiat Oncol Biol Phys 2020 Dec 1;108(5):1204-1217. Epub 2020 Aug 6 doi: 10.1016/j.ijrobp.2020.07.2324. PMID: 32768563
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Medulloblastoma: Distinctive Histo-Molecular Correlation with Clinical Profile, Radiologic Characteristics, and Surgical Outcome.
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
Re-irradiation for children with recurrent medulloblastoma in Toronto, Canada: a 20-year experience.
Tsang DS, Sarhan N, Ramaswamy V, Nobre L, Yee R, Taylor MD, Hawkins C, Bartels U, Huang A, Tabori U, Hodgson DC, Bouffet E, Laperriere N
J Neurooncol 2019 Oct;145(1):107-114. Epub 2019 Aug 29 doi: 10.1007/s11060-019-03272-2. PMID: 31468270

Diagnosis

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
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Re-irradiation for children with recurrent medulloblastoma in Toronto, Canada: a 20-year experience.
Tsang DS, Sarhan N, Ramaswamy V, Nobre L, Yee R, Taylor MD, Hawkins C, Bartels U, Huang A, Tabori U, Hodgson DC, Bouffet E, Laperriere N
J Neurooncol 2019 Oct;145(1):107-114. Epub 2019 Aug 29 doi: 10.1007/s11060-019-03272-2. PMID: 31468270
DNA methylation profiling is a method of choice for molecular verification of pediatric WNT-activated medulloblastomas.
Korshunov A, Sahm F, Zheludkova O, Golanov A, Stichel D, Schrimpf D, Ryzhova M, Potapov A, Habel A, Meyer J, Lichter P, Jones DTW, von Deimling A, Pfister SM, Kool M
Neuro Oncol 2019 Feb 14;21(2):214-221. doi: 10.1093/neuonc/noy155. PMID: 30252101Free PMC Article

Therapy

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 alterations of TP53 and OTX2 indicate increased risk of relapse in WNT medulloblastomas.
Goschzik T, Mynarek M, Doerner E, Schenk A, Spier I, Warmuth-Metz M, Bison B, Obrecht D, Struve N, Kortmann RD, Schmid M, Aretz S, Rutkowski S, Pietsch T
Acta Neuropathol 2022 Dec;144(6):1143-1156. Epub 2022 Oct 1 doi: 10.1007/s00401-022-02505-5. PMID: 36181537Free PMC Article
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
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Re-irradiation for children with recurrent medulloblastoma in Toronto, Canada: a 20-year experience.
Tsang DS, Sarhan N, Ramaswamy V, Nobre L, Yee R, Taylor MD, Hawkins C, Bartels U, Huang A, Tabori U, Hodgson DC, Bouffet E, Laperriere N
J Neurooncol 2019 Oct;145(1):107-114. Epub 2019 Aug 29 doi: 10.1007/s11060-019-03272-2. PMID: 31468270

Prognosis

Genetic alterations of TP53 and OTX2 indicate increased risk of relapse in WNT medulloblastomas.
Goschzik T, Mynarek M, Doerner E, Schenk A, Spier I, Warmuth-Metz M, Bison B, Obrecht D, Struve N, Kortmann RD, Schmid M, Aretz S, Rutkowski S, Pietsch T
Acta Neuropathol 2022 Dec;144(6):1143-1156. Epub 2022 Oct 1 doi: 10.1007/s00401-022-02505-5. PMID: 36181537Free PMC Article
Recurrent Wnt medulloblastoma treated with marrow-ablative chemotherapy and autologous hematopoietic progenitor cell rescue: a dual case report and review of the literature.
Harris MK, Shatara M, Funk Z, Stanek J, Boué DR, Jones J, Finlay JL, Abdelbaki MS
Childs Nerv Syst 2022 Feb;38(2):465-472. Epub 2021 May 4 doi: 10.1007/s00381-021-05197-6. PMID: 33948723
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
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Medulloblastoma: Distinctive Histo-Molecular Correlation with Clinical Profile, Radiologic Characteristics, and Surgical Outcome.
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

Clinical prediction guides

Exclusive Hyperfractionated Radiation Therapy and Reduced Boost Volume for Standard-Risk Medulloblastoma: Pooled Analysis of the 2 French Multicentric Studies MSFOP98 and MSFOP 2007 and Correlation With Molecular Subgroups.
Carrie C, Kieffer V, Figarella-Branger D, Masliah-Planchon J, Bolle S, Bernier V, Laprie A, Supiot S, Leseur J, Habrand JL, Alapetite C, Kerr C, Dufour C, Claude L, Chapet S, Huchet A, Bondiau PY, Escande A, Truc G, Nguyen TD, Pasteuris C, Vigneron C, Muracciole X, Bourdeaut F, Appay R, Dubray B, Colin C, Ferlay C, Dussart S, Chabaud S, Padovani L; French Group of Pediatric Radiotherapy (GFRP); French Society of Pediatric Cancers (SFCE)
Int J Radiat Oncol Biol Phys 2020 Dec 1;108(5):1204-1217. Epub 2020 Aug 6 doi: 10.1016/j.ijrobp.2020.07.2324. PMID: 32768563
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
Molecular stratifications, biomarker candidates and new therapeutic options in current medulloblastoma treatment approaches.
Menyhárt O, Győrffy B
Cancer Metastasis Rev 2020 Mar;39(1):211-233. doi: 10.1007/s10555-020-09854-1. PMID: 31970590Free PMC Article
Medulloblastoma: Distinctive Histo-Molecular Correlation with Clinical Profile, Radiologic Characteristics, and Surgical Outcome.
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

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