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MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019
Medulloblastoma is one of the most common types of brain and other CNS tumors among the pediatric population in the US. Molecularly-defined brain tumor histopathologies—including medulloblastoma subtypes—were incorporated into US cancer registry reporting for individuals with brain tumors beginning...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10260202/ http://dx.doi.org/10.1093/neuonc/noad073.251 |
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author | Ostrom, Quinn T Price, Mackenzie Neff, Corey Cioffi, Gino Waite, Kristin A Kruchko, Carol Barnholtz-Sloan, Jill S |
author_facet | Ostrom, Quinn T Price, Mackenzie Neff, Corey Cioffi, Gino Waite, Kristin A Kruchko, Carol Barnholtz-Sloan, Jill S |
author_sort | Ostrom, Quinn T |
collection | PubMed |
description | Medulloblastoma is one of the most common types of brain and other CNS tumors among the pediatric population in the US. Molecularly-defined brain tumor histopathologies—including medulloblastoma subtypes—were incorporated into US cancer registry reporting for individuals with brain tumors beginning in 2018. We assessed the epidemiology and overall survival (OS) patterns for medulloblastoma in children and adolescents, highlighting molecularly-defined subtypes. Children and adolescents (0-19 years) that were histopathologically diagnosed with medulloblastoma from 2018-2019 and had brain molecular marker data were identified within the Commission on Cancer’s National Cancer Database (NCDB) and Central Brain Tumor Registry of the United States databases, which combines data from CDC’s National Program of Cancer Registries (NPCR) and NCI’s Surveillance, Epidemiology, and End Results (SEER) Programs. Incidence rates per 100,000 population with 95% confidence intervals (95CI) were estimated for histopathologies with ≥16 cases. One-year OS was estimated using NCDB data for histopathologies with ≥50 cases with follow-up through 2020 using Kaplan-Meier methods. There were 601 cases of histopathologically-confirmed medulloblastoma diagnosed in children and adolescents from 2018-2019, 39.6% of which had available molecular subtype information. Overall incidence of medulloblastoma was 0.37 (95CI=0.34-0.40). Incidence of medulloblastoma subtypes was 0.05 (95CI=0.04-0.06) for SHH-activated & TP53wt, 0.02 (95CI=0.01-0.02) for WNT-activated, 0.08 (95CI=0.06-0.09) for nonWNT/nonSHH, while SHH-activated & TP53mut was too rare to calculate incidence. One-year OS for all medulloblastoma was 96.6% (95CI=92.1%-100.0%). One-year OS for nonWNT/nonSHH was 96.6% (95CI=92.8%-100.0%), and for SHH-activated & TP53wt was 93.7% (95CI=87.1%-100.0%). Other subtypes occurred too rarely to calculate survival. Our findings provide the initial US epidemiological estimates for molecularly-defined medulloblastoma histopathologies in children and adolescents. As improvements in collection completeness lead to increased molecular subtype availability, the robustness of these estimates will increase. Collection of these data are essential for understanding the epidemiology of this important childhood and adolescent brain tumor. |
format | Online Article Text |
id | pubmed-10260202 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-102602022023-06-13 MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 Ostrom, Quinn T Price, Mackenzie Neff, Corey Cioffi, Gino Waite, Kristin A Kruchko, Carol Barnholtz-Sloan, Jill S Neuro Oncol Final Category: Medulloblastomas - MDB Medulloblastoma is one of the most common types of brain and other CNS tumors among the pediatric population in the US. Molecularly-defined brain tumor histopathologies—including medulloblastoma subtypes—were incorporated into US cancer registry reporting for individuals with brain tumors beginning in 2018. We assessed the epidemiology and overall survival (OS) patterns for medulloblastoma in children and adolescents, highlighting molecularly-defined subtypes. Children and adolescents (0-19 years) that were histopathologically diagnosed with medulloblastoma from 2018-2019 and had brain molecular marker data were identified within the Commission on Cancer’s National Cancer Database (NCDB) and Central Brain Tumor Registry of the United States databases, which combines data from CDC’s National Program of Cancer Registries (NPCR) and NCI’s Surveillance, Epidemiology, and End Results (SEER) Programs. Incidence rates per 100,000 population with 95% confidence intervals (95CI) were estimated for histopathologies with ≥16 cases. One-year OS was estimated using NCDB data for histopathologies with ≥50 cases with follow-up through 2020 using Kaplan-Meier methods. There were 601 cases of histopathologically-confirmed medulloblastoma diagnosed in children and adolescents from 2018-2019, 39.6% of which had available molecular subtype information. Overall incidence of medulloblastoma was 0.37 (95CI=0.34-0.40). Incidence of medulloblastoma subtypes was 0.05 (95CI=0.04-0.06) for SHH-activated & TP53wt, 0.02 (95CI=0.01-0.02) for WNT-activated, 0.08 (95CI=0.06-0.09) for nonWNT/nonSHH, while SHH-activated & TP53mut was too rare to calculate incidence. One-year OS for all medulloblastoma was 96.6% (95CI=92.1%-100.0%). One-year OS for nonWNT/nonSHH was 96.6% (95CI=92.8%-100.0%), and for SHH-activated & TP53wt was 93.7% (95CI=87.1%-100.0%). Other subtypes occurred too rarely to calculate survival. Our findings provide the initial US epidemiological estimates for molecularly-defined medulloblastoma histopathologies in children and adolescents. As improvements in collection completeness lead to increased molecular subtype availability, the robustness of these estimates will increase. Collection of these data are essential for understanding the epidemiology of this important childhood and adolescent brain tumor. Oxford University Press 2023-06-12 /pmc/articles/PMC10260202/ http://dx.doi.org/10.1093/neuonc/noad073.251 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Final Category: Medulloblastomas - MDB Ostrom, Quinn T Price, Mackenzie Neff, Corey Cioffi, Gino Waite, Kristin A Kruchko, Carol Barnholtz-Sloan, Jill S MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title | MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title_full | MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title_fullStr | MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title_full_unstemmed | MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title_short | MDB-18. MOLECULARLY-DEFINED MEDULLOBLASTOMA: POPULATION-LEVEL PATTERNS IN THE UNITED STATES, 2018-2019 |
title_sort | mdb-18. molecularly-defined medulloblastoma: population-level patterns in the united states, 2018-2019 |
topic | Final Category: Medulloblastomas - MDB |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10260202/ http://dx.doi.org/10.1093/neuonc/noad073.251 |
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