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Related Experiment Videos

Familial risks in nervous system tumors.

Kari Hemminki1, Xinjun Li

  • 1Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany. kari.hemminki@cnt.ki.se

Cancer Epidemiology, Biomarkers & Prevention : a Publication of the American Association for Cancer Research, Cosponsored by the American Society of Preventive Oncology
|December 4, 2003
PubMed
Summary

Familial risk for nervous system tumors, particularly brain tumors like meningioma and astrocytoma, is elevated. Hemangioblastoma showed the strongest familial link, suggesting potential unknown hereditary conditions.

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Area of Science:

  • Neuro-oncology
  • Cancer genetics
  • Epidemiology

Background:

  • Nervous system tumors can have familial predispositions.
  • Understanding familial risk is crucial for genetic counseling and early detection.

Purpose of the Study:

  • To investigate the familial risk of nervous system tumors using a nationwide Swedish database.
  • To identify specific tumor types with increased hereditary risk.

Main Methods:

  • Utilized the Swedish Family-Cancer Database (1961-2000).
  • Analyzed nearly 11,000 nervous system tumor cases in offspring (0-68 years).
  • Calculated standardized incidence ratios (SIRs) for familial risk based on parental and sibling diagnoses.

Main Results:

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  • Increased familial risk (SIRs) observed for meningioma, astrocytoma, and hemangioblastoma.
  • Offspring SIRs were 3.06 (parental meningioma), 2.19 (parental astrocytoma), and 165 (parental hemangioblastoma).
  • Sibling SIRs were 4.41 (meningioma), 3.20 (astrocytoma), and 61 (hemangioblastoma).
  • Hemangioblastoma showed complete familial determination (kappa=1.00).
  • Age-specific analysis of familial astrocytoma revealed three incidence peaks.

Conclusions:

  • Specific brain tumor histologies, including meningioma, astrocytoma, and hemangioblastoma, demonstrate significant familial risk.
  • The strong familial link for hemangioblastoma aligns with known syndromes like von Hippel-Lindau disease.
  • Many familial cases of astrocytoma and meningioma may indicate previously unrecognized hereditary cancer syndromes.