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A population-based study of familial central nervous system hemangioblastomas

Hemminki, K LU ; Li, Xinjun LU and Collins, V P (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
publishing date
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
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
2019-02-20 11:46:01
@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},
  keyword      = {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},
  volume       = {20},
  year         = {2001},
}