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

Eljas Alanen, Sanna Heikkinen, Riikka Nurminen, Matti Nykter, Hannu Haapasalo, Elli Hirvonen, Janne Pitkäniemi, Kirsi J. Rautajoki

Research output: Contribution to journalArticleScientificpeer-review


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.

Original languageEnglish
Article numbervdad008
Number of pages10
JournalNeuro-Oncology Advances
Issue number1
Publication statusPublished - Feb 2023
Publication typeA1 Journal article-refereed


  • Brain tumor
  • epidemiology
  • familial aggregation
  • standardized incidence ratio
  • tumor syndrome

Publication forum classification

  • Publication forum level 0

ASJC Scopus subject areas

  • Surgery
  • Oncology
  • Clinical Neurology


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