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Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy

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Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy. / Tonn, Svenja; Korshunov, Andrey; Obrecht, Denise; Sill, Martin; Spohn, Michael; von Hoff, Katja; Milde, Till; Pietsch, Torsten; Goschzik, Tobias; Bison, Brigitte; Juhnke, Björn-Ole; Struve, Nina; Sturm, Dominik; Sahm, Felix; Bockmayr, Michael; Friedrich, Carsten; von Bueren, André O; Gerber, Nicolas U; Benesch, Martin; Jones, David T W; Kool, Marcel; Wefers, Annika K; Schüller, Ulrich; Pfister, Stefan M; Rutkowski, Stefan; Mynarek, Martin.

in: NEURO-ONCOLOGY, Jahrgang 25, Nr. 8, 03.08.2023, S. 1518-1529.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Tonn, S, Korshunov, A, Obrecht, D, Sill, M, Spohn, M, von Hoff, K, Milde, T, Pietsch, T, Goschzik, T, Bison, B, Juhnke, B-O, Struve, N, Sturm, D, Sahm, F, Bockmayr, M, Friedrich, C, von Bueren, AO, Gerber, NU, Benesch, M, Jones, DTW, Kool, M, Wefers, AK, Schüller, U, Pfister, SM, Rutkowski, S & Mynarek, M 2023, 'Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy', NEURO-ONCOLOGY, Jg. 25, Nr. 8, S. 1518-1529. https://doi.org/10.1093/neuonc/noad027

APA

Tonn, S., Korshunov, A., Obrecht, D., Sill, M., Spohn, M., von Hoff, K., Milde, T., Pietsch, T., Goschzik, T., Bison, B., Juhnke, B-O., Struve, N., Sturm, D., Sahm, F., Bockmayr, M., Friedrich, C., von Bueren, A. O., Gerber, N. U., Benesch, M., ... Mynarek, M. (2023). Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy. NEURO-ONCOLOGY, 25(8), 1518-1529. https://doi.org/10.1093/neuonc/noad027

Vancouver

Tonn S, Korshunov A, Obrecht D, Sill M, Spohn M, von Hoff K et al. Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy. NEURO-ONCOLOGY. 2023 Aug 3;25(8):1518-1529. https://doi.org/10.1093/neuonc/noad027

Bibtex

@article{4cd0a83456f6435291bfe74badaa638f,
title = "Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy",
abstract = "BACKGROUND: The prognostic impact of clinical risk factors and DNA methylation patterns in sonic hedgehog (SHH)-activated early childhood desmoplastic/nodular medulloblastoma (DMB) or medulloblastoma with extensive nodularity (MBEN) were evaluated to better identify patients at risk for relapse.METHODS: One hundred and forty-four patients with DMB (n = 99) or MBEN (n = 45) aged <5 years and treated with radiation-sparing approaches, including intraventricular methotrexate in 132 patients were evaluated.RESULTS: Patients with DMB had less favorable 5-year progression-free survival than MBEN (5y-PFS, 71% [DMB] vs. 93% [MBEN]). Patients aged >3 years were associated with more unfavorable 5y-PFS (47% [>3 years] vs. 85% [<1 year] vs. 84% [1-3 years]). DNA methylation profiles available (n = 78) were reclassified according to the 2021 WHO classification into SHH-1 (n = 39), SHH-2 (n = 38), and SHH-3 (n = 1). Hierarchical clustering delineated 2 subgroups among SHH-2: SHH-2a (n = 19) and SHH-2b (n = 19). Patients with SHH-2b medulloblastoma were older, predominantly displayed DMB histology, and were more often located in the cerebellar hemispheres. Chromosome 9q losses were more frequent in SHH-2b, while few chromosomal alterations were observed in SHH-2a. SHH-2b medulloblastoma carried a significantly increased relapse risk (5y-PFS: 58% [SHH-2b] vs. 83% [SHH-1] vs. 95% [SHH-2a]). Subclassification of SHH-2 with key clinical and cytogenetic characteristics was confirmed using 2 independent cohorts (total n = 188). Gene mutation analysis revealed a correlation of SHH-2a with SMO mutations.CONCLUSIONS: These data suggest further heterogeneity within early childhood SHH-DMB/MBEN: SHH-2 splits into a very low-risk group SHH-2a enriched for MBEN histology and SMO mutations, and SHH-2b comprising older DMB patients with a higher risk of relapse.",
author = "Svenja Tonn and Andrey Korshunov and Denise Obrecht and Martin Sill and Michael Spohn and {von Hoff}, Katja and Till Milde and Torsten Pietsch and Tobias Goschzik and Brigitte Bison and Bj{\"o}rn-Ole Juhnke and Nina Struve and Dominik Sturm and Felix Sahm and Michael Bockmayr and Carsten Friedrich and {von Bueren}, {Andr{\'e} O} and Gerber, {Nicolas U} and Martin Benesch and Jones, {David T W} and Marcel Kool and Wefers, {Annika K} and Ulrich Sch{\"u}ller and Pfister, {Stefan M} and Stefan Rutkowski and Martin Mynarek",
note = "{\textcopyright} The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.",
year = "2023",
month = aug,
day = "3",
doi = "10.1093/neuonc/noad027",
language = "English",
volume = "25",
pages = "1518--1529",
journal = "NEURO-ONCOLOGY",
issn = "1522-8517",
publisher = "Oxford University Press",
number = "8",

}

RIS

TY - JOUR

T1 - Risk prediction in early childhood sonic hedgehog medulloblastoma treated with radiation-avoiding chemotherapy

AU - Tonn, Svenja

AU - Korshunov, Andrey

AU - Obrecht, Denise

AU - Sill, Martin

AU - Spohn, Michael

AU - von Hoff, Katja

AU - Milde, Till

AU - Pietsch, Torsten

AU - Goschzik, Tobias

AU - Bison, Brigitte

AU - Juhnke, Björn-Ole

AU - Struve, Nina

AU - Sturm, Dominik

AU - Sahm, Felix

AU - Bockmayr, Michael

AU - Friedrich, Carsten

AU - von Bueren, André O

AU - Gerber, Nicolas U

AU - Benesch, Martin

AU - Jones, David T W

AU - Kool, Marcel

AU - Wefers, Annika K

AU - Schüller, Ulrich

AU - Pfister, Stefan M

AU - Rutkowski, Stefan

AU - Mynarek, Martin

N1 - © The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

PY - 2023/8/3

Y1 - 2023/8/3

N2 - BACKGROUND: The prognostic impact of clinical risk factors and DNA methylation patterns in sonic hedgehog (SHH)-activated early childhood desmoplastic/nodular medulloblastoma (DMB) or medulloblastoma with extensive nodularity (MBEN) were evaluated to better identify patients at risk for relapse.METHODS: One hundred and forty-four patients with DMB (n = 99) or MBEN (n = 45) aged <5 years and treated with radiation-sparing approaches, including intraventricular methotrexate in 132 patients were evaluated.RESULTS: Patients with DMB had less favorable 5-year progression-free survival than MBEN (5y-PFS, 71% [DMB] vs. 93% [MBEN]). Patients aged >3 years were associated with more unfavorable 5y-PFS (47% [>3 years] vs. 85% [<1 year] vs. 84% [1-3 years]). DNA methylation profiles available (n = 78) were reclassified according to the 2021 WHO classification into SHH-1 (n = 39), SHH-2 (n = 38), and SHH-3 (n = 1). Hierarchical clustering delineated 2 subgroups among SHH-2: SHH-2a (n = 19) and SHH-2b (n = 19). Patients with SHH-2b medulloblastoma were older, predominantly displayed DMB histology, and were more often located in the cerebellar hemispheres. Chromosome 9q losses were more frequent in SHH-2b, while few chromosomal alterations were observed in SHH-2a. SHH-2b medulloblastoma carried a significantly increased relapse risk (5y-PFS: 58% [SHH-2b] vs. 83% [SHH-1] vs. 95% [SHH-2a]). Subclassification of SHH-2 with key clinical and cytogenetic characteristics was confirmed using 2 independent cohorts (total n = 188). Gene mutation analysis revealed a correlation of SHH-2a with SMO mutations.CONCLUSIONS: These data suggest further heterogeneity within early childhood SHH-DMB/MBEN: SHH-2 splits into a very low-risk group SHH-2a enriched for MBEN histology and SMO mutations, and SHH-2b comprising older DMB patients with a higher risk of relapse.

AB - BACKGROUND: The prognostic impact of clinical risk factors and DNA methylation patterns in sonic hedgehog (SHH)-activated early childhood desmoplastic/nodular medulloblastoma (DMB) or medulloblastoma with extensive nodularity (MBEN) were evaluated to better identify patients at risk for relapse.METHODS: One hundred and forty-four patients with DMB (n = 99) or MBEN (n = 45) aged <5 years and treated with radiation-sparing approaches, including intraventricular methotrexate in 132 patients were evaluated.RESULTS: Patients with DMB had less favorable 5-year progression-free survival than MBEN (5y-PFS, 71% [DMB] vs. 93% [MBEN]). Patients aged >3 years were associated with more unfavorable 5y-PFS (47% [>3 years] vs. 85% [<1 year] vs. 84% [1-3 years]). DNA methylation profiles available (n = 78) were reclassified according to the 2021 WHO classification into SHH-1 (n = 39), SHH-2 (n = 38), and SHH-3 (n = 1). Hierarchical clustering delineated 2 subgroups among SHH-2: SHH-2a (n = 19) and SHH-2b (n = 19). Patients with SHH-2b medulloblastoma were older, predominantly displayed DMB histology, and were more often located in the cerebellar hemispheres. Chromosome 9q losses were more frequent in SHH-2b, while few chromosomal alterations were observed in SHH-2a. SHH-2b medulloblastoma carried a significantly increased relapse risk (5y-PFS: 58% [SHH-2b] vs. 83% [SHH-1] vs. 95% [SHH-2a]). Subclassification of SHH-2 with key clinical and cytogenetic characteristics was confirmed using 2 independent cohorts (total n = 188). Gene mutation analysis revealed a correlation of SHH-2a with SMO mutations.CONCLUSIONS: These data suggest further heterogeneity within early childhood SHH-DMB/MBEN: SHH-2 splits into a very low-risk group SHH-2a enriched for MBEN histology and SMO mutations, and SHH-2b comprising older DMB patients with a higher risk of relapse.

U2 - 10.1093/neuonc/noad027

DO - 10.1093/neuonc/noad027

M3 - SCORING: Journal article

C2 - 36715306

VL - 25

SP - 1518

EP - 1529

JO - NEURO-ONCOLOGY

JF - NEURO-ONCOLOGY

SN - 1522-8517

IS - 8

ER -