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Familial colorectal adenocarcinoma from the Swedish family‐cancer database
Author(s) -
Hemminki Kari,
Li Xinjun
Publication year - 2001
Publication title -
international journal of cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.475
H-Index - 234
eISSN - 1097-0215
pISSN - 0020-7136
DOI - 10.1002/ijc.1533
Subject(s) - medicine , colorectal cancer , penetrance , adenocarcinoma , family history , offspring , cancer , incidence (geometry) , oncology , family aggregation , cancer registry , disease , biology , genetics , pregnancy , gene , physics , optics , phenotype
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. © 2001 Wiley‐Liss, Inc.