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Medulloblastoma non-WNT/non-SHH group 4

MedGen UID:
1377534
•Concept ID:
C4330666
•
Neoplastic Process
Synonyms: medulloblastoma non-WNT/non-SHH group 4; Medulloblastoma, Non-WNT/Non-SHH, Group 4
 
Monarch Initiative: MONDO:0956967

Definition

Medulloblastoma not associated with activation of the WNT pathway or sonic hedgehog (SHH) pathway. MYC amplifications are absent. TP53 mutations are absent. Chromosome 17 abnormalities may be present. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMedulloblastoma non-WNT/non-SHH group 4

Professional guidelines

PubMed

Medulloblastoma in adults: an analysis of clinico-pathological, molecular and treatment factors.
Franchino F, Morra I, Forni M, Bertero L, Zanini C, Roveta F, Ricardi U, Mantovani C, Carpaneto A, Migliore E, Pellerino A, Ferrio F, Cassoni P, Garbossa D, Soffietti R, Rudà R
J Neurosurg Sci 2024 Jun;68(3):260-269. Epub 2021 Nov 11 doi: 10.23736/S0390-5616.21.05548-X. PMID: 34763393
Postoperative radiotherapy timing, molecular subgroups and treatment outcomes of Thai pediatric patients with medulloblastoma.
Jaruthien T, Nantavithya C, Santisukwongchote S, Shuangshoti S, Techavichit P, Sosothikul D, Amornfa J, Shotelersuk K
PLoS One 2023;18(1):e0271778. Epub 2023 Jan 17 doi: 10.1371/journal.pone.0271778. PMID: 36649280Free PMC Article
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

Recent clinical studies

Therapy

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
Prognostic effect of whole chromosomal aberration signatures in standard-risk, non-WNT/non-SHH medulloblastoma: a retrospective, molecular analysis of the HIT-SIOP PNET 4 trial.
Goschzik T, Schwalbe EC, Hicks D, Smith A, Zur Muehlen A, Figarella-Branger D, Doz F, Rutkowski S, Lannering B, Pietsch T, Clifford SC
Lancet Oncol 2018 Dec;19(12):1602-1616. Epub 2018 Nov 1 doi: 10.1016/S1470-2045(18)30532-1. PMID: 30392813Free PMC Article
Medulloblastoma therapy generates risk of a poorly-prognostic H3 wild-type subgroup of diffuse intrinsic pontine glioma: a report from the International DIPG Registry.
Gits HC, Anderson M, Stallard S, Pratt D, Zon B, Howell C, Kumar-Sinha C, Vats P, Kasaian K, Polan D, Matuszak M, Spratt DE, Leonard M, Qin T, Zhao L, Leach J, Chaney B, Escorza NY, Hendershot J, Jones B, Fuller C, Leary S, Bartels U, Bouffet E, Yock TI, Robertson P, Mody R, Venneti S, Chinnaiyan AM, Fouladi M, Gottardo NG, Koschmann C
Acta Neuropathol Commun 2018 Jul 26;6(1):67. doi: 10.1186/s40478-018-0570-9. PMID: 30049282Free PMC Article
Multicenter pilot study of radiochemotherapy as first-line treatment for adults with medulloblastoma (NOA-07).
Beier D, Proescholdt M, Reinert C, Pietsch T, Jones DTW, Pfister SM, Hattingen E, Seidel C, Dirven L, Luerding R, Reijneveld J, Warmuth-Metz M, Bonsanto M, Bremer M, Combs SE, Rieken S, Herrlinger U, Kuntze H, Mayer-Steinacker R, Moskopp D, Schneider T, Beringer A, Schlegel U, Stummer W, Welker H, Weyerbrock A, Paulsen F, Rutkowski S, Weller M, Wick W, Kortmann RD, Bogdahn U, Hau P
Neuro Oncol 2018 Feb 19;20(3):400-410. doi: 10.1093/neuonc/nox155. PMID: 29016837Free PMC Article
Imaging Biomarkers for Adult Medulloblastomas: Genetic Entities May Be Identified by Their MR Imaging Radiophenotype.
Keil VC, Warmuth-Metz M, Reh C, Enkirch SJ, Reinert C, Beier D, Jones DTW, Pietsch T, Schild HH, Hattingen E, Hau P
AJNR Am J Neuroradiol 2017 Oct;38(10):1892-1898. Epub 2017 Aug 10 doi: 10.3174/ajnr.A5313. PMID: 28798218Free PMC Article

Prognosis

Transcriptome analysis stratifies second-generation non-WNT/non-SHH medulloblastoma subgroups into clinically tractable subtypes.
Korshunov A, Okonechnikov K, Schrimpf D, Tonn S, Mynarek M, Koster J, Sievers P, Milde T, Sahm F, Jones DTW, von Deimling A, Pfister SM, Kool M
Acta Neuropathol 2023 Jun;145(6):829-842. Epub 2023 Apr 24 doi: 10.1007/s00401-023-02575-z. PMID: 37093271
Molecular characterisation defines clinically-actionable heterogeneity within Group 4 medulloblastoma and improves disease risk-stratification.
Goddard J, Castle J, Southworth E, Fletcher A, Crosier S, Martin-Guerrero I, García-Ariza M, Navajas A, Masliah-Planchon J, Bourdeaut F, Dufour C, Ayrault O, Goschzik T, Pietsch T, Sill M, Pfister SM, Rutkowski S, Richardson S, Hill RM, Williamson D, Bailey S, Schwalbe EC, Clifford SC, Hicks D
Acta Neuropathol 2023 May;145(5):651-666. Epub 2023 Apr 4 doi: 10.1007/s00401-023-02566-0. PMID: 37014508Free PMC Article
Molecular Stratification of Medulloblastoma: Clinical Outcomes and Therapeutic Interventions.
Sursal T, Ronecker JS, Dicpinigaitis AJ, Mohan AL, Tobias ME, Gandhi CD, Jhanwar-Uniyal M
Anticancer Res 2022 May;42(5):2225-2239. doi: 10.21873/anticanres.15703. PMID: 35489737
Clinically Tractable Outcome Prediction of Non-WNT/Non-SHH Medulloblastoma Based on TPD52 IHC in a Multicohort Study.
Delaidelli A, Dunham C, Santi M, Negri GL, Triscott J, Zheludkova O, Golanov A, Ryzhova M, Okonechnikov K, Schrimpf D, Stichel D, Ellison DW, von Deimling A, Kool M, Pfister SM, Ramaswamy V, Korshunov A, Taylor MD, Sorensen PH
Clin Cancer Res 2022 Jan 1;28(1):116-128. Epub 2021 Oct 26 doi: 10.1158/1078-0432.CCR-21-2057. PMID: 34702771
Molecular markers and potential therapeutic targets in non-WNT/non-SHH (group 3 and group 4) medulloblastomas.
Menyhárt O, Giangaspero F, Győrffy B
J Hematol Oncol 2019 Mar 15;12(1):29. doi: 10.1186/s13045-019-0712-y. PMID: 30876441Free PMC Article

Clinical prediction guides

Transcriptome analysis stratifies second-generation non-WNT/non-SHH medulloblastoma subgroups into clinically tractable subtypes.
Korshunov A, Okonechnikov K, Schrimpf D, Tonn S, Mynarek M, Koster J, Sievers P, Milde T, Sahm F, Jones DTW, von Deimling A, Pfister SM, Kool M
Acta Neuropathol 2023 Jun;145(6):829-842. Epub 2023 Apr 24 doi: 10.1007/s00401-023-02575-z. PMID: 37093271
Molecular characterisation defines clinically-actionable heterogeneity within Group 4 medulloblastoma and improves disease risk-stratification.
Goddard J, Castle J, Southworth E, Fletcher A, Crosier S, Martin-Guerrero I, García-Ariza M, Navajas A, Masliah-Planchon J, Bourdeaut F, Dufour C, Ayrault O, Goschzik T, Pietsch T, Sill M, Pfister SM, Rutkowski S, Richardson S, Hill RM, Williamson D, Bailey S, Schwalbe EC, Clifford SC, Hicks D
Acta Neuropathol 2023 May;145(5):651-666. Epub 2023 Apr 4 doi: 10.1007/s00401-023-02566-0. PMID: 37014508Free PMC Article
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
Clinically Tractable Outcome Prediction of Non-WNT/Non-SHH Medulloblastoma Based on TPD52 IHC in a Multicohort Study.
Delaidelli A, Dunham C, Santi M, Negri GL, Triscott J, Zheludkova O, Golanov A, Ryzhova M, Okonechnikov K, Schrimpf D, Stichel D, Ellison DW, von Deimling A, Kool M, Pfister SM, Ramaswamy V, Korshunov A, Taylor MD, Sorensen PH
Clin Cancer Res 2022 Jan 1;28(1):116-128. Epub 2021 Oct 26 doi: 10.1158/1078-0432.CCR-21-2057. PMID: 34702771
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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