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Risk of pancreatic cancer in families with Lynch syndrome.
JAMA 2009; 302(16):1790-5JAMA

Abstract

CONTEXT

Lynch syndrome is an inherited cause of colorectal cancer caused by mutations of DNA mismatch repair (MMR) genes. A number of extracolonic tumors have been associated with the disorder, including pancreatic cancer; however, the risk of pancreatic cancer in Lynch syndrome is uncertain and not quantified.

OBJECTIVE

To estimate pancreatic cancer risk in families with germline MMR gene mutations.

DESIGN, SETTING, AND PATIENTS

Cancer histories of probands and their relatives were evaluated in MMR gene mutation carriers in the familial cancer registries of the Dana-Farber Cancer Institute (n = 80), Boston, Massachusetts, and University of Michigan Comprehensive Cancer Center (n = 67), Ann Arbor, Michigan. Families enrolled before the study start date (June 2008) were eligible. Age-specific cumulative risks and hazard ratio estimates of pancreatic cancer risk were calculated and compared with the general population using modified segregation analysis, with correction for ascertainment.

MAIN OUTCOME MEASURES

Age-specific cumulative risks and hazard ratio estimates of pancreatic cancer risk.

RESULTS

Data on 6342 individuals from 147 families with MMR gene mutations were analyzed. Thirty-one families (21.1%) reported at least 1 case of pancreatic cancer. Forty-seven pancreatic cancers were reported (21 men and 26 women), with no sex-related difference in age of diagnosis (51.5 vs 56.5 years for men and women, respectively). The cumulative risk of pancreatic cancer in these families with gene mutations was 1.31% (95% confidence interval [CI], 0.31%-2.32%) up to age 50 years and 3.68% (95% CI, 1.45%-5.88%) up to age 70 years, which represents an 8.6-fold increase (95% CI, 4.7-15.7) compared with the general population.

CONCLUSIONS

Among 147 families with germline MMR gene mutations, the risk of pancreatic cancer was increased compared with the US population. Individuals with MMR gene mutations and a family history of pancreatic cancer are appropriate to include in studies to further define the risk of premalignant and malignant pancreatic neoplasms and potential benefits and limitations of surveillance.

Authors+Show Affiliations

Department of Internal Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19861671

Citation

Kastrinos, Fay, et al. "Risk of Pancreatic Cancer in Families With Lynch Syndrome." JAMA, vol. 302, no. 16, 2009, pp. 1790-5.
Kastrinos F, Mukherjee B, Tayob N, et al. Risk of pancreatic cancer in families with Lynch syndrome. JAMA. 2009;302(16):1790-5.
Kastrinos, F., Mukherjee, B., Tayob, N., Wang, F., Sparr, J., Raymond, V. M., ... Syngal, S. (2009). Risk of pancreatic cancer in families with Lynch syndrome. JAMA, 302(16), pp. 1790-5. doi:10.1001/jama.2009.1529.
Kastrinos F, et al. Risk of Pancreatic Cancer in Families With Lynch Syndrome. JAMA. 2009 Oct 28;302(16):1790-5. PubMed PMID: 19861671.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Risk of pancreatic cancer in families with Lynch syndrome. AU - Kastrinos,Fay, AU - Mukherjee,Bhramar, AU - Tayob,Nabihah, AU - Wang,Fei, AU - Sparr,Jennifer, AU - Raymond,Victoria M, AU - Bandipalliam,Prathap, AU - Stoffel,Elena M, AU - Gruber,Stephen B, AU - Syngal,Sapna, PY - 2009/10/29/entrez PY - 2009/10/29/pubmed PY - 2009/11/3/medline SP - 1790 EP - 5 JF - JAMA JO - JAMA VL - 302 IS - 16 N2 - CONTEXT: Lynch syndrome is an inherited cause of colorectal cancer caused by mutations of DNA mismatch repair (MMR) genes. A number of extracolonic tumors have been associated with the disorder, including pancreatic cancer; however, the risk of pancreatic cancer in Lynch syndrome is uncertain and not quantified. OBJECTIVE: To estimate pancreatic cancer risk in families with germline MMR gene mutations. DESIGN, SETTING, AND PATIENTS: Cancer histories of probands and their relatives were evaluated in MMR gene mutation carriers in the familial cancer registries of the Dana-Farber Cancer Institute (n = 80), Boston, Massachusetts, and University of Michigan Comprehensive Cancer Center (n = 67), Ann Arbor, Michigan. Families enrolled before the study start date (June 2008) were eligible. Age-specific cumulative risks and hazard ratio estimates of pancreatic cancer risk were calculated and compared with the general population using modified segregation analysis, with correction for ascertainment. MAIN OUTCOME MEASURES: Age-specific cumulative risks and hazard ratio estimates of pancreatic cancer risk. RESULTS: Data on 6342 individuals from 147 families with MMR gene mutations were analyzed. Thirty-one families (21.1%) reported at least 1 case of pancreatic cancer. Forty-seven pancreatic cancers were reported (21 men and 26 women), with no sex-related difference in age of diagnosis (51.5 vs 56.5 years for men and women, respectively). The cumulative risk of pancreatic cancer in these families with gene mutations was 1.31% (95% confidence interval [CI], 0.31%-2.32%) up to age 50 years and 3.68% (95% CI, 1.45%-5.88%) up to age 70 years, which represents an 8.6-fold increase (95% CI, 4.7-15.7) compared with the general population. CONCLUSIONS: Among 147 families with germline MMR gene mutations, the risk of pancreatic cancer was increased compared with the US population. Individuals with MMR gene mutations and a family history of pancreatic cancer are appropriate to include in studies to further define the risk of premalignant and malignant pancreatic neoplasms and potential benefits and limitations of surveillance. SN - 1538-3598 UR - https://www.unboundmedicine.com/medline/citation/19861671/Risk_of_pancreatic_cancer_in_families_with_Lynch_syndrome_ L2 - https://jamanetwork.com/journals/jama/fullarticle/10.1001/jama.2009.1529 DB - PRIME DP - Unbound Medicine ER -