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Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives

BACKGROUND: Central nervous system (CNS) tumors are a heterogeneous group of tumors that include several aggressive malignancies with a high mortality rate. This study aimed to evaluate the familial relative risk of CNS tumors in family members of early-onset index cases (probands) in and between di...

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Autores principales: Alanen, Eljas, Heikkinen, Sanna, Nurminen, Riikka, Nykter, Matti, Haapasalo, Hannu, Hirvonen, Elli, Pitkäniemi, Janne, Rautajoki, Kirsi J
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10025807/
https://www.ncbi.nlm.nih.gov/pubmed/36950216
http://dx.doi.org/10.1093/noajnl/vdad008
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author Alanen, Eljas
Heikkinen, Sanna
Nurminen, Riikka
Nykter, Matti
Haapasalo, Hannu
Hirvonen, Elli
Pitkäniemi, Janne
Rautajoki, Kirsi J
author_facet Alanen, Eljas
Heikkinen, Sanna
Nurminen, Riikka
Nykter, Matti
Haapasalo, Hannu
Hirvonen, Elli
Pitkäniemi, Janne
Rautajoki, Kirsi J
author_sort Alanen, Eljas
collection PubMed
description BACKGROUND: Central nervous system (CNS) tumors are a heterogeneous group of tumors that include several aggressive malignancies with a high mortality rate. This study aimed to evaluate the familial relative risk of CNS tumors in family members of early-onset index cases (probands) in and between diffuse glioma, non-diffuse glioma, meningioma, and other CNS tumors. METHODS: We retrieved tumor data from the Finnish cancer registry and familial relationships data from the population information system. We ascertained 5408 probands diagnosed with primary CNS tumors (age ≤40 years) between 1970 and 2012 in Finland. We report the standardized incidence ratios as a measure of familial aggregation using Poisson regression. RESULTS: The risk of early-onset diffuse glioma increased among siblings of probands with the same tumor [SIR 3.85, 95% confidence interval (CI): 1.66–7.59], with association mainly returning to grade 2–3 diffuse gliomas. Early-onset other CNS tumors were associated with an increased risk of other CNS tumors, early-onset meningioma, and late-onset diffuse glioma in 1st-degree relatives. The elevated risk of other CNS tumors was largely caused by schwannomas (SIR 59.44, 95% CI: 27.18–112.84 for 1st-degree relatives) and associated with neurofibromatosis. No tumor syndrome was associated with an increased risk of diffuse gliomas. CONCLUSIONS: The early onset of grade 2–3 diffuse gliomas is associated with an increased risk of similar tumor entities. Early-onset schwannomas dramatically increase CNS tumor risk with a broader tumor-type profile. In future studies, it would be important to identify the underlying shared hereditary factors that contribute to the development of familial diffuse gliomas.
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spelling pubmed-100258072023-03-21 Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives Alanen, Eljas Heikkinen, Sanna Nurminen, Riikka Nykter, Matti Haapasalo, Hannu Hirvonen, Elli Pitkäniemi, Janne Rautajoki, Kirsi J Neurooncol Adv Basic and Translational Investigations BACKGROUND: Central nervous system (CNS) tumors are a heterogeneous group of tumors that include several aggressive malignancies with a high mortality rate. This study aimed to evaluate the familial relative risk of CNS tumors in family members of early-onset index cases (probands) in and between diffuse glioma, non-diffuse glioma, meningioma, and other CNS tumors. METHODS: We retrieved tumor data from the Finnish cancer registry and familial relationships data from the population information system. We ascertained 5408 probands diagnosed with primary CNS tumors (age ≤40 years) between 1970 and 2012 in Finland. We report the standardized incidence ratios as a measure of familial aggregation using Poisson regression. RESULTS: The risk of early-onset diffuse glioma increased among siblings of probands with the same tumor [SIR 3.85, 95% confidence interval (CI): 1.66–7.59], with association mainly returning to grade 2–3 diffuse gliomas. Early-onset other CNS tumors were associated with an increased risk of other CNS tumors, early-onset meningioma, and late-onset diffuse glioma in 1st-degree relatives. The elevated risk of other CNS tumors was largely caused by schwannomas (SIR 59.44, 95% CI: 27.18–112.84 for 1st-degree relatives) and associated with neurofibromatosis. No tumor syndrome was associated with an increased risk of diffuse gliomas. CONCLUSIONS: The early onset of grade 2–3 diffuse gliomas is associated with an increased risk of similar tumor entities. Early-onset schwannomas dramatically increase CNS tumor risk with a broader tumor-type profile. In future studies, it would be important to identify the underlying shared hereditary factors that contribute to the development of familial diffuse gliomas. Oxford University Press 2023-02-01 /pmc/articles/PMC10025807/ /pubmed/36950216 http://dx.doi.org/10.1093/noajnl/vdad008 Text en © The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology. https://creativecommons.org/licenses/by/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Basic and Translational Investigations
Alanen, Eljas
Heikkinen, Sanna
Nurminen, Riikka
Nykter, Matti
Haapasalo, Hannu
Hirvonen, Elli
Pitkäniemi, Janne
Rautajoki, Kirsi J
Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title_full Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title_fullStr Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title_full_unstemmed Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title_short Early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
title_sort early-onset grade 2-3 diffuse gliomas and schwannomas increase the risk of central nervous system tumors among the patients’ relatives
topic Basic and Translational Investigations
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10025807/
https://www.ncbi.nlm.nih.gov/pubmed/36950216
http://dx.doi.org/10.1093/noajnl/vdad008
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