Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome

Niloy Jewel Samadder, Ken Robert Smith, Jathine Wong, Alun Thomas, Heidi Hanson, Kenneth Boucher, Cathryn Kopituch, Lisa A. Cannon-Albright, Randall W. Burt, Karen Curtin

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Importance: The data describing cancer risks associated with Lynch syndrome are variable. Objectives: To quantify the prevalence of families that fulfill the Amsterdam I or II criteria for Lynch syndrome in the Utah population and investigate the risk of colonic and extracolonic cancers in family members and their relatives. Design, Setting, and Participants: In a population-based study, 202 families with Amsterdam I and II criteria–positive pedigrees in the Utah Population Database were identified. Of these, all cancer diagnoses in members of families with Amsterdam criteria and their first-degree, second-degree, and first-cousin relatives were located through linkage to the Utah Cancer Registry. The study was conducted from May 1 to June 30, 2016. Main Outcomes and Measures: Standardized morbidity ratios (SMRs) were estimated by comparing the observed rates of cancer in relatives with population-expected rates estimated internally from the Utah Population Database. Results: A total of 202 families meeting Amsterdam criteria for Lynch syndrome accounted for 2.6% of all colorectal cancers in the state; of these, 59 met both the Amsterdam I and Amsterdam II criteria. Cancers observed in significant excess in the first-degree relatives of Amsterdam criteria pedigrees included colorectal (SMR, 10.10; 95% CI, 9.43-10.81), endometrial (SMR, 5.89; 95% CI, 5.09-6.78), stomach (SMR, 2.90; 95% CI, 2.02-4.03), small intestine (SMR, 7.72; 95% CI, 5.17-11.08), prostate (SMR, 1.94; 95% CI, 1.73-2.17), kidney (SMR, 3.22; 95% CI, 2.45-4.16), urinary bladder (SMR, 1.62; 95% CI, 1.22-2.12), thyroid (SMR, 2.26; 95% CI, 1.55-3.17), and non-Hodgkin lymphoma (SMR, 2.10; 95% CI, 1.64-2.65). Risks of colorectal and endometrial cancers were also found to be elevated in second-degree (SMR, 4.31; 95% CI, 3.98-4.65 and SMR, 2.70; 95% CI, 2.30-3.14, respectively) and first-cousin (SMR, 1.85; 95% CI, 1.70-2.00 and SMR, 1.50; 95% CI, 1.29-1.73, respectively) relatives of families with Amsterdam criteria. Conclusions and Relevance: In this population-based study of cancer risk in families fulfilling the Amsterdam criteria, many of the cancers previously reported to be associated with Lynch syndrome were observed, several previously unreported cancer associations were noted, and the risk of colorectal and endometrial cancer were markedly increased in first-, second-, and even third-degree relatives of these families. This study provides clinicians with population-based, unbiased data to counsel members of families meeting the Amsterdam criteria regarding their elevated risks of cancer and the importance of cancer screening.

Original languageEnglish (US)
Pages (from-to)1697-1701
Number of pages5
JournalJAMA Oncology
Volume3
Issue number12
DOIs
StatePublished - Dec 1 2017

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Hereditary Nonpolyposis Colorectal Neoplasms
Morbidity
Neoplasms
Population
Colorectal Neoplasms
Pedigree
Endometrial Neoplasms
Lynch Syndrome II
Databases
Early Detection of Cancer
Non-Hodgkin's Lymphoma
Colonic Neoplasms

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Samadder, N. J., Smith, K. R., Wong, J., Thomas, A., Hanson, H., Boucher, K., ... Curtin, K. (2017). Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome. JAMA Oncology, 3(12), 1697-1701. https://doi.org/10.1001/jamaoncol.2017.0769

Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome. / Samadder, Niloy Jewel; Smith, Ken Robert; Wong, Jathine; Thomas, Alun; Hanson, Heidi; Boucher, Kenneth; Kopituch, Cathryn; Cannon-Albright, Lisa A.; Burt, Randall W.; Curtin, Karen.

In: JAMA Oncology, Vol. 3, No. 12, 01.12.2017, p. 1697-1701.

Research output: Contribution to journalArticle

Samadder, NJ, Smith, KR, Wong, J, Thomas, A, Hanson, H, Boucher, K, Kopituch, C, Cannon-Albright, LA, Burt, RW & Curtin, K 2017, 'Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome', JAMA Oncology, vol. 3, no. 12, pp. 1697-1701. https://doi.org/10.1001/jamaoncol.2017.0769
Samadder, Niloy Jewel ; Smith, Ken Robert ; Wong, Jathine ; Thomas, Alun ; Hanson, Heidi ; Boucher, Kenneth ; Kopituch, Cathryn ; Cannon-Albright, Lisa A. ; Burt, Randall W. ; Curtin, Karen. / Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome. In: JAMA Oncology. 2017 ; Vol. 3, No. 12. pp. 1697-1701.
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abstract = "Importance: The data describing cancer risks associated with Lynch syndrome are variable. Objectives: To quantify the prevalence of families that fulfill the Amsterdam I or II criteria for Lynch syndrome in the Utah population and investigate the risk of colonic and extracolonic cancers in family members and their relatives. Design, Setting, and Participants: In a population-based study, 202 families with Amsterdam I and II criteria–positive pedigrees in the Utah Population Database were identified. Of these, all cancer diagnoses in members of families with Amsterdam criteria and their first-degree, second-degree, and first-cousin relatives were located through linkage to the Utah Cancer Registry. The study was conducted from May 1 to June 30, 2016. Main Outcomes and Measures: Standardized morbidity ratios (SMRs) were estimated by comparing the observed rates of cancer in relatives with population-expected rates estimated internally from the Utah Population Database. Results: A total of 202 families meeting Amsterdam criteria for Lynch syndrome accounted for 2.6{\%} of all colorectal cancers in the state; of these, 59 met both the Amsterdam I and Amsterdam II criteria. Cancers observed in significant excess in the first-degree relatives of Amsterdam criteria pedigrees included colorectal (SMR, 10.10; 95{\%} CI, 9.43-10.81), endometrial (SMR, 5.89; 95{\%} CI, 5.09-6.78), stomach (SMR, 2.90; 95{\%} CI, 2.02-4.03), small intestine (SMR, 7.72; 95{\%} CI, 5.17-11.08), prostate (SMR, 1.94; 95{\%} CI, 1.73-2.17), kidney (SMR, 3.22; 95{\%} CI, 2.45-4.16), urinary bladder (SMR, 1.62; 95{\%} CI, 1.22-2.12), thyroid (SMR, 2.26; 95{\%} CI, 1.55-3.17), and non-Hodgkin lymphoma (SMR, 2.10; 95{\%} CI, 1.64-2.65). Risks of colorectal and endometrial cancers were also found to be elevated in second-degree (SMR, 4.31; 95{\%} CI, 3.98-4.65 and SMR, 2.70; 95{\%} CI, 2.30-3.14, respectively) and first-cousin (SMR, 1.85; 95{\%} CI, 1.70-2.00 and SMR, 1.50; 95{\%} CI, 1.29-1.73, respectively) relatives of families with Amsterdam criteria. Conclusions and Relevance: In this population-based study of cancer risk in families fulfilling the Amsterdam criteria, many of the cancers previously reported to be associated with Lynch syndrome were observed, several previously unreported cancer associations were noted, and the risk of colorectal and endometrial cancer were markedly increased in first-, second-, and even third-degree relatives of these families. This study provides clinicians with population-based, unbiased data to counsel members of families meeting the Amsterdam criteria regarding their elevated risks of cancer and the importance of cancer screening.",
author = "Samadder, {Niloy Jewel} and Smith, {Ken Robert} and Jathine Wong and Alun Thomas and Heidi Hanson and Kenneth Boucher and Cathryn Kopituch and Cannon-Albright, {Lisa A.} and Burt, {Randall W.} and Karen Curtin",
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T1 - Cancer Risk in Families Fulfilling the Amsterdam Criteria for Lynch Syndrome

AU - Samadder, Niloy Jewel

AU - Smith, Ken Robert

AU - Wong, Jathine

AU - Thomas, Alun

AU - Hanson, Heidi

AU - Boucher, Kenneth

AU - Kopituch, Cathryn

AU - Cannon-Albright, Lisa A.

AU - Burt, Randall W.

AU - Curtin, Karen

PY - 2017/12/1

Y1 - 2017/12/1

N2 - Importance: The data describing cancer risks associated with Lynch syndrome are variable. Objectives: To quantify the prevalence of families that fulfill the Amsterdam I or II criteria for Lynch syndrome in the Utah population and investigate the risk of colonic and extracolonic cancers in family members and their relatives. Design, Setting, and Participants: In a population-based study, 202 families with Amsterdam I and II criteria–positive pedigrees in the Utah Population Database were identified. Of these, all cancer diagnoses in members of families with Amsterdam criteria and their first-degree, second-degree, and first-cousin relatives were located through linkage to the Utah Cancer Registry. The study was conducted from May 1 to June 30, 2016. Main Outcomes and Measures: Standardized morbidity ratios (SMRs) were estimated by comparing the observed rates of cancer in relatives with population-expected rates estimated internally from the Utah Population Database. Results: A total of 202 families meeting Amsterdam criteria for Lynch syndrome accounted for 2.6% of all colorectal cancers in the state; of these, 59 met both the Amsterdam I and Amsterdam II criteria. Cancers observed in significant excess in the first-degree relatives of Amsterdam criteria pedigrees included colorectal (SMR, 10.10; 95% CI, 9.43-10.81), endometrial (SMR, 5.89; 95% CI, 5.09-6.78), stomach (SMR, 2.90; 95% CI, 2.02-4.03), small intestine (SMR, 7.72; 95% CI, 5.17-11.08), prostate (SMR, 1.94; 95% CI, 1.73-2.17), kidney (SMR, 3.22; 95% CI, 2.45-4.16), urinary bladder (SMR, 1.62; 95% CI, 1.22-2.12), thyroid (SMR, 2.26; 95% CI, 1.55-3.17), and non-Hodgkin lymphoma (SMR, 2.10; 95% CI, 1.64-2.65). Risks of colorectal and endometrial cancers were also found to be elevated in second-degree (SMR, 4.31; 95% CI, 3.98-4.65 and SMR, 2.70; 95% CI, 2.30-3.14, respectively) and first-cousin (SMR, 1.85; 95% CI, 1.70-2.00 and SMR, 1.50; 95% CI, 1.29-1.73, respectively) relatives of families with Amsterdam criteria. Conclusions and Relevance: In this population-based study of cancer risk in families fulfilling the Amsterdam criteria, many of the cancers previously reported to be associated with Lynch syndrome were observed, several previously unreported cancer associations were noted, and the risk of colorectal and endometrial cancer were markedly increased in first-, second-, and even third-degree relatives of these families. This study provides clinicians with population-based, unbiased data to counsel members of families meeting the Amsterdam criteria regarding their elevated risks of cancer and the importance of cancer screening.

AB - Importance: The data describing cancer risks associated with Lynch syndrome are variable. Objectives: To quantify the prevalence of families that fulfill the Amsterdam I or II criteria for Lynch syndrome in the Utah population and investigate the risk of colonic and extracolonic cancers in family members and their relatives. Design, Setting, and Participants: In a population-based study, 202 families with Amsterdam I and II criteria–positive pedigrees in the Utah Population Database were identified. Of these, all cancer diagnoses in members of families with Amsterdam criteria and their first-degree, second-degree, and first-cousin relatives were located through linkage to the Utah Cancer Registry. The study was conducted from May 1 to June 30, 2016. Main Outcomes and Measures: Standardized morbidity ratios (SMRs) were estimated by comparing the observed rates of cancer in relatives with population-expected rates estimated internally from the Utah Population Database. Results: A total of 202 families meeting Amsterdam criteria for Lynch syndrome accounted for 2.6% of all colorectal cancers in the state; of these, 59 met both the Amsterdam I and Amsterdam II criteria. Cancers observed in significant excess in the first-degree relatives of Amsterdam criteria pedigrees included colorectal (SMR, 10.10; 95% CI, 9.43-10.81), endometrial (SMR, 5.89; 95% CI, 5.09-6.78), stomach (SMR, 2.90; 95% CI, 2.02-4.03), small intestine (SMR, 7.72; 95% CI, 5.17-11.08), prostate (SMR, 1.94; 95% CI, 1.73-2.17), kidney (SMR, 3.22; 95% CI, 2.45-4.16), urinary bladder (SMR, 1.62; 95% CI, 1.22-2.12), thyroid (SMR, 2.26; 95% CI, 1.55-3.17), and non-Hodgkin lymphoma (SMR, 2.10; 95% CI, 1.64-2.65). Risks of colorectal and endometrial cancers were also found to be elevated in second-degree (SMR, 4.31; 95% CI, 3.98-4.65 and SMR, 2.70; 95% CI, 2.30-3.14, respectively) and first-cousin (SMR, 1.85; 95% CI, 1.70-2.00 and SMR, 1.50; 95% CI, 1.29-1.73, respectively) relatives of families with Amsterdam criteria. Conclusions and Relevance: In this population-based study of cancer risk in families fulfilling the Amsterdam criteria, many of the cancers previously reported to be associated with Lynch syndrome were observed, several previously unreported cancer associations were noted, and the risk of colorectal and endometrial cancer were markedly increased in first-, second-, and even third-degree relatives of these families. This study provides clinicians with population-based, unbiased data to counsel members of families meeting the Amsterdam criteria regarding their elevated risks of cancer and the importance of cancer screening.

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