Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families

Yun Hee Choi, Lajmi Lakhal-Chaieb, Agnieszka Kröl, Bing Yu, Daniel Buchanan, Dennis Ahnen, Loic Le Marchand, Polly A. Newcomb, Aung Ko Win, Mark Jenkins, Noralane Morey Lindor, Laurent Briollais

Research output: Contribution to journalArticle

Abstract

Background: The risk of cancers is well characterized in Lynch syndrome (LS) families but has been less studied in familial colorectal cancer type X (FCCTX) families. Methods: In this article, we compare the risk estimates of first and second colorectal cancers (CRCs) in 168 FCTTX and 780 LS families recruited through the Colon Cancer Family Registry as well as the risk of cancer-related deaths and disease-free survival (DFS) after a first CRC. Our methodology is based on a survival analysis approach, developed specifically to model the occurrence of successive cancers (ie, first and second CRCs) in the presence of competing risk events (ie, death from any causes). Results: We found an excess risk of first and second CRC in individuals with LS compared to FCCTX family members. However, for an average age at first CRC of 60 years in FCCTX families and 50 years in LS families, the DFS rates were comparable in men but lower in women from FCCTX vs LS families, eg, 75.1% (95% confidence interval [CI] = 69.0% to 80.9%) vs 78.9% (95% CI = 76.3% to 81.3%) for the 10-year DFS. The 10-year risk of cancer-related death was higher in FCCTX families vs LS families, eg, 15.4% in men (95% CI = 10.9% to 19.8%) and 19.3% in women (95% CI = 13.6% to 24.7%) vs 8.9% (95% CI = 7.5% to 11.4%) and 8.7% (95% CI = 7.1% to 10.8%), respectively. Conclusions: Individuals with CRCs arising in the context of FCCTX do not experience the same improved DFS and overall survival of those with LS, and that difference may be relevant in management decisions.

Original languageEnglish (US)
Article numberdjy159
Pages (from-to)675-683
Number of pages9
JournalJournal of the National Cancer Institute
Volume111
Issue number7
DOIs
StatePublished - Jul 1 2019

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Hereditary Nonpolyposis Colorectal Neoplasms
Colorectal Neoplasms
Mortality
Neoplasms
Confidence Intervals
Disease-Free Survival
Second Primary Neoplasms
Survival Analysis
Colonic Neoplasms
Registries
Cause of Death

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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Choi, Y. H., Lakhal-Chaieb, L., Kröl, A., Yu, B., Buchanan, D., Ahnen, D., ... Briollais, L. (2019). Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families. Journal of the National Cancer Institute, 111(7), 675-683. [djy159]. https://doi.org/10.1093/jnci/djy159

Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families. / Choi, Yun Hee; Lakhal-Chaieb, Lajmi; Kröl, Agnieszka; Yu, Bing; Buchanan, Daniel; Ahnen, Dennis; Le Marchand, Loic; Newcomb, Polly A.; Win, Aung Ko; Jenkins, Mark; Lindor, Noralane Morey; Briollais, Laurent.

In: Journal of the National Cancer Institute, Vol. 111, No. 7, djy159, 01.07.2019, p. 675-683.

Research output: Contribution to journalArticle

Choi, YH, Lakhal-Chaieb, L, Kröl, A, Yu, B, Buchanan, D, Ahnen, D, Le Marchand, L, Newcomb, PA, Win, AK, Jenkins, M, Lindor, NM & Briollais, L 2019, 'Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families', Journal of the National Cancer Institute, vol. 111, no. 7, djy159, pp. 675-683. https://doi.org/10.1093/jnci/djy159
Choi, Yun Hee ; Lakhal-Chaieb, Lajmi ; Kröl, Agnieszka ; Yu, Bing ; Buchanan, Daniel ; Ahnen, Dennis ; Le Marchand, Loic ; Newcomb, Polly A. ; Win, Aung Ko ; Jenkins, Mark ; Lindor, Noralane Morey ; Briollais, Laurent. / Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families. In: Journal of the National Cancer Institute. 2019 ; Vol. 111, No. 7. pp. 675-683.
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title = "Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families",
abstract = "Background: The risk of cancers is well characterized in Lynch syndrome (LS) families but has been less studied in familial colorectal cancer type X (FCCTX) families. Methods: In this article, we compare the risk estimates of first and second colorectal cancers (CRCs) in 168 FCTTX and 780 LS families recruited through the Colon Cancer Family Registry as well as the risk of cancer-related deaths and disease-free survival (DFS) after a first CRC. Our methodology is based on a survival analysis approach, developed specifically to model the occurrence of successive cancers (ie, first and second CRCs) in the presence of competing risk events (ie, death from any causes). Results: We found an excess risk of first and second CRC in individuals with LS compared to FCCTX family members. However, for an average age at first CRC of 60 years in FCCTX families and 50 years in LS families, the DFS rates were comparable in men but lower in women from FCCTX vs LS families, eg, 75.1{\%} (95{\%} confidence interval [CI] = 69.0{\%} to 80.9{\%}) vs 78.9{\%} (95{\%} CI = 76.3{\%} to 81.3{\%}) for the 10-year DFS. The 10-year risk of cancer-related death was higher in FCCTX families vs LS families, eg, 15.4{\%} in men (95{\%} CI = 10.9{\%} to 19.8{\%}) and 19.3{\%} in women (95{\%} CI = 13.6{\%} to 24.7{\%}) vs 8.9{\%} (95{\%} CI = 7.5{\%} to 11.4{\%}) and 8.7{\%} (95{\%} CI = 7.1{\%} to 10.8{\%}), respectively. Conclusions: Individuals with CRCs arising in the context of FCCTX do not experience the same improved DFS and overall survival of those with LS, and that difference may be relevant in management decisions.",
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T1 - Risks of Colorectal Cancer and Cancer-Related Mortality in Familial Colorectal Cancer Type X and Lynch Syndrome Families

AU - Choi, Yun Hee

AU - Lakhal-Chaieb, Lajmi

AU - Kröl, Agnieszka

AU - Yu, Bing

AU - Buchanan, Daniel

AU - Ahnen, Dennis

AU - Le Marchand, Loic

AU - Newcomb, Polly A.

AU - Win, Aung Ko

AU - Jenkins, Mark

AU - Lindor, Noralane Morey

AU - Briollais, Laurent

PY - 2019/7/1

Y1 - 2019/7/1

N2 - Background: The risk of cancers is well characterized in Lynch syndrome (LS) families but has been less studied in familial colorectal cancer type X (FCCTX) families. Methods: In this article, we compare the risk estimates of first and second colorectal cancers (CRCs) in 168 FCTTX and 780 LS families recruited through the Colon Cancer Family Registry as well as the risk of cancer-related deaths and disease-free survival (DFS) after a first CRC. Our methodology is based on a survival analysis approach, developed specifically to model the occurrence of successive cancers (ie, first and second CRCs) in the presence of competing risk events (ie, death from any causes). Results: We found an excess risk of first and second CRC in individuals with LS compared to FCCTX family members. However, for an average age at first CRC of 60 years in FCCTX families and 50 years in LS families, the DFS rates were comparable in men but lower in women from FCCTX vs LS families, eg, 75.1% (95% confidence interval [CI] = 69.0% to 80.9%) vs 78.9% (95% CI = 76.3% to 81.3%) for the 10-year DFS. The 10-year risk of cancer-related death was higher in FCCTX families vs LS families, eg, 15.4% in men (95% CI = 10.9% to 19.8%) and 19.3% in women (95% CI = 13.6% to 24.7%) vs 8.9% (95% CI = 7.5% to 11.4%) and 8.7% (95% CI = 7.1% to 10.8%), respectively. Conclusions: Individuals with CRCs arising in the context of FCCTX do not experience the same improved DFS and overall survival of those with LS, and that difference may be relevant in management decisions.

AB - Background: The risk of cancers is well characterized in Lynch syndrome (LS) families but has been less studied in familial colorectal cancer type X (FCCTX) families. Methods: In this article, we compare the risk estimates of first and second colorectal cancers (CRCs) in 168 FCTTX and 780 LS families recruited through the Colon Cancer Family Registry as well as the risk of cancer-related deaths and disease-free survival (DFS) after a first CRC. Our methodology is based on a survival analysis approach, developed specifically to model the occurrence of successive cancers (ie, first and second CRCs) in the presence of competing risk events (ie, death from any causes). Results: We found an excess risk of first and second CRC in individuals with LS compared to FCCTX family members. However, for an average age at first CRC of 60 years in FCCTX families and 50 years in LS families, the DFS rates were comparable in men but lower in women from FCCTX vs LS families, eg, 75.1% (95% confidence interval [CI] = 69.0% to 80.9%) vs 78.9% (95% CI = 76.3% to 81.3%) for the 10-year DFS. The 10-year risk of cancer-related death was higher in FCCTX families vs LS families, eg, 15.4% in men (95% CI = 10.9% to 19.8%) and 19.3% in women (95% CI = 13.6% to 24.7%) vs 8.9% (95% CI = 7.5% to 11.4%) and 8.7% (95% CI = 7.1% to 10.8%), respectively. Conclusions: Individuals with CRCs arising in the context of FCCTX do not experience the same improved DFS and overall survival of those with LS, and that difference may be relevant in management decisions.

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