A population-based study of familial central nervous system hemangioblastomas
(2001) In Neuroepidemiology 20(4). p.61-257- Abstract
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... (More)
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.
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- author
- Hemminki, K LU ; Li, Xinjun LU and Collins, V P
- publishing date
- 2001-10
- type
- Contribution to journal
- publication status
- published
- 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
- in
- Neuroepidemiology
- volume
- 20
- issue
- 4
- pages
- 5 pages
- publisher
- Karger
- external identifiers
-
- scopus:0035742904
- pmid:11684902
- ISSN
- 0251-5350
- DOI
- 10.1159/000054799
- language
- English
- LU publication?
- no
- id
- 8f28f8ef-6efc-43d4-b16b-68804a294b1f
- date added to LUP
- 2019-01-30 12:13:14
- date last changed
- 2024-08-20 09:26:05
@article{8f28f8ef-6efc-43d4-b16b-68804a294b1f, abstract = {{<p>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.</p>}}, author = {{Hemminki, K and Li, Xinjun and Collins, V P}}, issn = {{0251-5350}}, 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}}, language = {{eng}}, number = {{4}}, pages = {{61--257}}, publisher = {{Karger}}, series = {{Neuroepidemiology}}, title = {{A population-based study of familial central nervous system hemangioblastomas}}, url = {{http://dx.doi.org/10.1159/000054799}}, doi = {{10.1159/000054799}}, volume = {{20}}, year = {{2001}}, }