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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...

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Autores principales: Ostrom, Quinn T, Price, Mackenzie, Neff, Corey, Cioffi, Gino, Waite, Kristin A, Kruchko, Carol, Barnholtz-Sloan, Jill S
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
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.
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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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