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Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review

OBJECTIVE: To describe disease outcomes including overall survival and relapse patterns by subgroup in young pediatric patients treated for medulloblastoma with a radiation-sparing approach. METHODS: Retrospective analysis of clinical outcomes includes treatment, relapse, and salvage therapy and lat...

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Autores principales: Ronsley, Rebecca, Triscott, Joanna, Stanek, Joseph, Rassekh, S. Rod, Lum, Amy, Cheng, Sylvia, Goddard, Karen, McConnell, Dina, Strahlendorf, Caron, Singhal, Ash, Finlay, Jonathan L., Yip, Stephen, Dunham, Christopher, Hukin, Juliette
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10390609/
https://www.ncbi.nlm.nih.gov/pubmed/37022464
http://dx.doi.org/10.1007/s00381-023-05918-z
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author Ronsley, Rebecca
Triscott, Joanna
Stanek, Joseph
Rassekh, S. Rod
Lum, Amy
Cheng, Sylvia
Goddard, Karen
McConnell, Dina
Strahlendorf, Caron
Singhal, Ash
Finlay, Jonathan L.
Yip, Stephen
Dunham, Christopher
Hukin, Juliette
author_facet Ronsley, Rebecca
Triscott, Joanna
Stanek, Joseph
Rassekh, S. Rod
Lum, Amy
Cheng, Sylvia
Goddard, Karen
McConnell, Dina
Strahlendorf, Caron
Singhal, Ash
Finlay, Jonathan L.
Yip, Stephen
Dunham, Christopher
Hukin, Juliette
author_sort Ronsley, Rebecca
collection PubMed
description OBJECTIVE: To describe disease outcomes including overall survival and relapse patterns by subgroup in young pediatric patients treated for medulloblastoma with a radiation-sparing approach. METHODS: Retrospective analysis of clinical outcomes includes treatment, relapse, and salvage therapy and late effects in children treated for medulloblastoma with a radiation-sparing approach at British Columbia Children’s Hospital (BCCH) between 2000 and 2020. RESULTS: There were 30 patients (median age 2.8 years, 60% male) treated for medulloblastoma with a radiation-sparing approach at BCCH. Subgroups included Sonic Hedgehog (SHH) (n = 14), group 3 (n = 7), group 4 (n = 6), and indeterminate status (n = 3). Three- and 5-year event-free survival (EFS) were 49.0% (30.2–65.4%) and 42.0% (24.2–58.9%) and overall survival (OS) 66.0% (95% CI 46.0–80.1%) and 62.5% (95% CI 42.5 and 77.2%), respectively, with a median follow-up of 9.5 years. Relapse occurred in 12/25 patients following a complete response, of whom six (group 4: n = 4; group 3: n = 1; unknown: n = 1) were successfully salvaged with craniospinal axis (CSA) RT and remain alive at a median follow-up of 7 years. Disease/treatment-related morbidity included endocrinopathies (n = 8), hearing loss n = 16), and neurocognitive abnormalities (n = 9). CONCLUSIONS: This radiation sparing treatment approach for young patients with medulloblastoma resulted in a durable cure in most patients with SHH subgroup medulloblastoma. In those patients with groups 3 and 4 medulloblastoma, relapse rates were high; however, most group 4 patients were salvaged with RT.
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spelling pubmed-103906092023-08-02 Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review Ronsley, Rebecca Triscott, Joanna Stanek, Joseph Rassekh, S. Rod Lum, Amy Cheng, Sylvia Goddard, Karen McConnell, Dina Strahlendorf, Caron Singhal, Ash Finlay, Jonathan L. Yip, Stephen Dunham, Christopher Hukin, Juliette Childs Nerv Syst Original Article OBJECTIVE: To describe disease outcomes including overall survival and relapse patterns by subgroup in young pediatric patients treated for medulloblastoma with a radiation-sparing approach. METHODS: Retrospective analysis of clinical outcomes includes treatment, relapse, and salvage therapy and late effects in children treated for medulloblastoma with a radiation-sparing approach at British Columbia Children’s Hospital (BCCH) between 2000 and 2020. RESULTS: There were 30 patients (median age 2.8 years, 60% male) treated for medulloblastoma with a radiation-sparing approach at BCCH. Subgroups included Sonic Hedgehog (SHH) (n = 14), group 3 (n = 7), group 4 (n = 6), and indeterminate status (n = 3). Three- and 5-year event-free survival (EFS) were 49.0% (30.2–65.4%) and 42.0% (24.2–58.9%) and overall survival (OS) 66.0% (95% CI 46.0–80.1%) and 62.5% (95% CI 42.5 and 77.2%), respectively, with a median follow-up of 9.5 years. Relapse occurred in 12/25 patients following a complete response, of whom six (group 4: n = 4; group 3: n = 1; unknown: n = 1) were successfully salvaged with craniospinal axis (CSA) RT and remain alive at a median follow-up of 7 years. Disease/treatment-related morbidity included endocrinopathies (n = 8), hearing loss n = 16), and neurocognitive abnormalities (n = 9). CONCLUSIONS: This radiation sparing treatment approach for young patients with medulloblastoma resulted in a durable cure in most patients with SHH subgroup medulloblastoma. In those patients with groups 3 and 4 medulloblastoma, relapse rates were high; however, most group 4 patients were salvaged with RT. Springer Berlin Heidelberg 2023-04-06 2023 /pmc/articles/PMC10390609/ /pubmed/37022464 http://dx.doi.org/10.1007/s00381-023-05918-z Text en © Crown 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Ronsley, Rebecca
Triscott, Joanna
Stanek, Joseph
Rassekh, S. Rod
Lum, Amy
Cheng, Sylvia
Goddard, Karen
McConnell, Dina
Strahlendorf, Caron
Singhal, Ash
Finlay, Jonathan L.
Yip, Stephen
Dunham, Christopher
Hukin, Juliette
Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title_full Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title_fullStr Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title_full_unstemmed Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title_short Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
title_sort outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10390609/
https://www.ncbi.nlm.nih.gov/pubmed/37022464
http://dx.doi.org/10.1007/s00381-023-05918-z
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