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Updated: Jun 12, 2026

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor
Published on: August 25, 2023
Familial risks in nervous system tumours: joint Nordic study
K Hemminki1, S Tretli, J H Olsen
1Division of Molecular Genetic Epidemiology, German Cancer Research Center (DKFZ), Heidelberg, Germany. k.hemminki@dkfz.de
Familial nervous system tumors show increased risks, especially for peripheral nerve tumors in multiplex families. These findings highlight the importance of genetic counseling for families with a history of nervous system cancers.
Area of Science:
- Neuro-oncology
- Cancer genetics
- Epidemiology
Background:
- Familial nervous system cancers are rare, with limited data on site-specific tumor risks.
- Understanding familial aggregation is crucial for genetic counseling and research.
Purpose of the Study:
- To analyze familial risks for nervous system tumors across different sites.
- To identify specific risk patterns in relation to affected relatives and tumor types.
Main Methods:
- Utilized data from five Nordic countries for a population-based study.
- Calculated standardized incidence ratios (SIRs) comparing offspring of affected versus non-affected relatives.
Main Results:
- Elevated SIRs observed for brain, spinal, and peripheral nerve tumors in offspring with affected relatives.
- Peripheral nerve tumors showed exceptionally high SIRs (up to 943.9) in multiplex families.
- Spinal tumor risks were significantly higher for offspring of early-onset cases.
Conclusions:
- Demonstrated site-, proband-, and age-specific risks for familial nervous system tumors.
- Results have implications for clinical genetic counseling and identifying genetic predispositions.
- Highlights the need for further research into the genetic underpinnings of familial nervous system cancers.
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