A population-based study of familial central nervous system hemangioblastomas

We used the nationwide Swedish Family-Cancer Database to analyze the risk f or central nervous system hemangioblastoma (HB) in offspring (0-61 years) o f 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 yon Hippel-Lindau (VHL) disease and prese nted with HBs, renal cell carcinomas, pheochromocytomas and insulomas in th e proband or other family members. Standardized incidence ratios (SIRs) wer e 600 for offspring HB by parental HB, and they were even high for the othe r 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 4 0-44 years, and it was twice as prevalent as the early-onset component. Bec ause there was no evidence of familial risks, this is suggested to be a spo radic form of HB. Copyright (C) 2001 S. Karger AG, Basel.