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Familial colorectal adenocarcinoma from the Swedish Family-Cancer Database

Hemminki, K LU and Li, Xinjun LU (2001) In International Journal of Cancer 94(5). p.8-743
Abstract

Familial risks for colorectal (CRC) adenocarcinoma were characterized from the Swedish Family-Cancer Database covering 9.6 million individuals, whose family relationships and cancers were obtained from registered sources, not sensitive to reporting or ascertainment bias. Cancer cases were retrieved from the Swedish Cancer Registry from years 1958-96. Standardized incidence ratios (SIRs) were calculated based on gender-, age-, period- and tumor type specific rates. A total of 4,794 and 67,925 CRCs were recorded in offspring and parents, respectively. For colon and rectal adenocarcinoma, the SIRs in offspring were 2.28 and 1.68 by parental CRC adenocarcinoma, giving attributable proportions of 6.45 and 3.31%, respectively. The SIR of CRC... (More)

Familial risks for colorectal (CRC) adenocarcinoma were characterized from the Swedish Family-Cancer Database covering 9.6 million individuals, whose family relationships and cancers were obtained from registered sources, not sensitive to reporting or ascertainment bias. Cancer cases were retrieved from the Swedish Cancer Registry from years 1958-96. Standardized incidence ratios (SIRs) were calculated based on gender-, age-, period- and tumor type specific rates. A total of 4,794 and 67,925 CRCs were recorded in offspring and parents, respectively. For colon and rectal adenocarcinoma, the SIRs in offspring were 2.28 and 1.68 by parental CRC adenocarcinoma, giving attributable proportions of 6.45 and 3.31%, respectively. The SIR of CRC was over 10 when both offspring and parents were diagnosed at a young age. The risk for parental CRC adenocarcinoma was over 100 when 2 or more children were affected. The risk in siblings was also very high when a parent was affected. The familial cancer sites that associated with CRC were those typical of hereditary nonpolyposis colorectal cancer (HNPCC). This is the largest study published on familial CRC and the only one reporting specifically on adenocarcinoma. The data suggest that HNPCC is the largest single disease entity among CRCs, probably accounting for less than 50% of familial CRC. Other familial components appear heterogeneous, characterized by incomplete penetrance, recessive mode of inheritance and few associated tumor sites.

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author
publishing date
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Contribution to journal
publication status
published
subject
keywords
Adenocarcinoma/genetics, Adolescent, Adult, Child, Child, Preschool, Colorectal Neoplasms/genetics, Colorectal Neoplasms, Hereditary Nonpolyposis/genetics, Databases as Topic, Family, Humans, Infant, Infant, Newborn, Middle Aged, Sweden
in
International Journal of Cancer
volume
94
issue
5
pages
6 pages
publisher
John Wiley & Sons
external identifiers
  • pmid:11745471
  • scopus:0035546004
ISSN
0020-7136
DOI
10.1002/ijc.1533
language
English
LU publication?
no
id
1b7724c6-baa4-4f14-8913-24f8f71711c7
date added to LUP
2019-01-30 12:12:06
date last changed
2019-11-20 05:36:35
@article{1b7724c6-baa4-4f14-8913-24f8f71711c7,
  abstract     = {<p>Familial risks for colorectal (CRC) adenocarcinoma were characterized from the Swedish Family-Cancer Database covering 9.6 million individuals, whose family relationships and cancers were obtained from registered sources, not sensitive to reporting or ascertainment bias. Cancer cases were retrieved from the Swedish Cancer Registry from years 1958-96. Standardized incidence ratios (SIRs) were calculated based on gender-, age-, period- and tumor type specific rates. A total of 4,794 and 67,925 CRCs were recorded in offspring and parents, respectively. For colon and rectal adenocarcinoma, the SIRs in offspring were 2.28 and 1.68 by parental CRC adenocarcinoma, giving attributable proportions of 6.45 and 3.31%, respectively. The SIR of CRC was over 10 when both offspring and parents were diagnosed at a young age. The risk for parental CRC adenocarcinoma was over 100 when 2 or more children were affected. The risk in siblings was also very high when a parent was affected. The familial cancer sites that associated with CRC were those typical of hereditary nonpolyposis colorectal cancer (HNPCC). This is the largest study published on familial CRC and the only one reporting specifically on adenocarcinoma. The data suggest that HNPCC is the largest single disease entity among CRCs, probably accounting for less than 50% of familial CRC. Other familial components appear heterogeneous, characterized by incomplete penetrance, recessive mode of inheritance and few associated tumor sites.</p>},
  author       = {Hemminki, K and Li, Xinjun},
  issn         = {0020-7136},
  language     = {eng},
  month        = {12},
  number       = {5},
  pages        = {8--743},
  publisher    = {John Wiley & Sons},
  series       = {International Journal of Cancer},
  title        = {Familial colorectal adenocarcinoma from the Swedish Family-Cancer Database},
  url          = {http://dx.doi.org/10.1002/ijc.1533},
  doi          = {10.1002/ijc.1533},
  volume       = {94},
  year         = {2001},
}