000182882 001__ 182882 000182882 005__ 20241230121009.0 000182882 0247_ $$2doi$$a10.1007/s00401-022-02522-4 000182882 0247_ $$2pmid$$apmid:36459208 000182882 0247_ $$2ISSN$$a0001-6322 000182882 0247_ $$2ISSN$$a1432-0533 000182882 0247_ $$2altmetric$$aaltmetric:139943480 000182882 037__ $$aDKFZ-2022-02988 000182882 041__ $$aEnglish 000182882 082__ $$a610 000182882 1001_ $$aMynarek, Martin$$b0 000182882 245__ $$aIdentification of low and very high-risk patients with non-WNT/non-SHH medulloblastoma by improved clinico-molecular stratification of the HIT2000 and I-HIT-MED cohorts. 000182882 260__ $$aHeidelberg$$bSpringer$$c2023 000182882 3367_ $$2DRIVER$$aarticle 000182882 3367_ $$2DataCite$$aOutput Types/Journal article 000182882 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1673260673_25367 000182882 3367_ $$2BibTeX$$aARTICLE 000182882 3367_ $$2ORCID$$aJOURNAL_ARTICLE 000182882 3367_ $$00$$2EndNote$$aJournal Article 000182882 500__ $$a#LA:B310# / 2023 Jan;145(1):97-112 000182882 520__ $$aMolecular groups of medulloblastoma (MB) are well established. Novel risk stratification parameters include Group 3/4 (non-WNT/non-SHH) methylation subgroups I-VIII or whole-chromosomal aberration (WCA) phenotypes. This study investigates the integration of clinical and molecular parameters to improve risk stratification of non-WNT/non-SHH MB. Non-WNT/non-SHH MB from the HIT2000 study and the HIT-MED registries were selected based on availability of DNA-methylation profiling data. MYC or MYCN amplification and WCA of chromosomes 7, 8, and 11 were inferred from methylation array-based copy number profiles. In total, 403 non-WNT/non-SHH MB were identified, 346/403 (86%) had a methylation class family Group 3/4 methylation score (classifier v11b6) ≥ 0.9, and 294/346 (73%) were included in the risk stratification modeling based on Group 3 or 4 score (v11b6) ≥ 0.8 and subgroup I-VIII score (mb_g34) ≥ 0.8. Group 3 MB (5y-PFS, survival estimation ± standard deviation: 41.4 ± 4.6%; 5y-OS: 48.8 ± 5.0%) showed poorer survival compared to Group 4 (5y-PFS: 68.2 ± 3.7%; 5y-OS: 84.8 ± 2.8%). Subgroups II (5y-PFS: 27.6 ± 8.2%) and III (5y-PFS: 37.5 ± 7.9%) showed the poorest and subgroup VI (5y-PFS: 76.6 ± 7.9%), VII (5y-PFS: 75.9 ± 7.2%), and VIII (5y-PFS: 66.6 ± 5.8%) the best survival. Multivariate analysis revealed subgroup in combination with WCA phenotype to best predict risk of progression and death. The integration of clinical (age, M and R status) and molecular (MYC/N, subgroup, WCA phenotype) variables identified a low-risk stratum with a 5y-PFS of 94 ± 5.7 and a very high-risk stratum with a 5y-PFS of 29 ± 6.1%. Validation in an international MB cohort confirmed the combined stratification scheme with 82.1 ± 6.0% 5y-PFS in the low and 47.5 ± 4.1% in very high-risk groups, and outperformed the clinical model. These newly identified clinico-molecular low-risk and very high-risk strata, accounting for 6%, and 21% of non-WNT/non-SHH MB patients, respectively, may improve future treatment stratification. 000182882 536__ $$0G:(DE-HGF)POF4-312$$a312 - Funktionelle und strukturelle Genomforschung (POF4-312)$$cPOF4-312$$fPOF IV$$x0 000182882 588__ $$aDataset connected to CrossRef, PubMed, , Journals: inrepo02.dkfz.de 000182882 650_7 $$2Other$$aGroup 3/4 000182882 650_7 $$2Other$$aMedulloblastoma 000182882 650_7 $$2Other$$aNon-WNT/non-SHH 000182882 650_7 $$2Other$$aRisk stratification 000182882 7001_ $$aObrecht, Denise$$b1 000182882 7001_ $$0P:(DE-He78)45440b44791309bd4b7dbb4f73333f9b$$aSill, Martin$$b2$$udkfz 000182882 7001_ $$0P:(DE-He78)a46a5b2a871859c8e2d63d2f8c666807$$aSturm, Dominik$$b3$$udkfz 000182882 7001_ $$aKloth-Stachnau, Katja$$b4 000182882 7001_ $$0P:(DE-He78)a23e88cc676489fe05be8c178ceaf58e$$aSelt, Florian$$b5$$udkfz 000182882 7001_ 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