A population-based study of familial central nervous system hemangioblastomas

Research output: Contribution to journalArticle


We used the nationwide Swedish Family-Cancer Database to analyze the risk for central nervous system hemangioblastoma (HB) in offspring (0-61 years) of parents with cancer. Eighty-three offspring were identified, and the age at onset showed a bimodal distribution. The early-onset component peaked at 25-29 years, was associated with von Hippel-Lindau (VHL) disease and presented with HBs, renal cell carcinomas, pheochromocytomas and insulomas in the proband or other family members. Standardized incidence ratios (SIRs) were 600 for offspring HB by parental HB, and they were even high for the other VHL-related tumors. Second tumors were common in this early-onset group, and the types were as expected in VHL. The late-onset component peaked at 40-44 years, and it was twice as prevalent as the early-onset component. Because there was no evidence of familial risks, this is suggested to be a sporadic form of HB.


External organisations
  • Karolinska Institutet
Research areas and keywords


  • Adolescent, Adult, Age of Onset, Central Nervous System Neoplasms/epidemiology, Child, Child, Preschool, Databases, Factual, Epidemiologic Studies, Female, Hemangioblastoma/epidemiology, Humans, Incidence, Infant, Infant, Newborn, Male, Middle Aged, Neoplasms/genetics, Pedigree, Risk Factors
Original languageEnglish
Pages (from-to)257-61
Number of pages5
Issue number4
Publication statusPublished - 2001 Oct
Publication categoryResearch
Externally publishedYes