Risk of Pancreatic Cancer in Families with Lynch Syndrome Defined

Researchers from several U.S. medical centers have reported that patients with Lynch syndrome have an 8.6-fold increase in the risk of pancreatic cancer compared with the general population. The details of this study appeared in the October 28, 2009 issue of the Journal of the American Medical Association.[1]

Lynch syndrome, also known as hereditary nonpolyposis colorectal cancer (HNPCC), results from inherited mutations in genes involved in DNA mismatch repair (MMR). HNPCC is an autosomal dominant disorder, which is characterized by early onset colorectal cancer and cancers of the endometrium, small intestine, ovary, hepatobiliary system, kidney, and ureter. Discovery of the genes involved in HNPCC has facilitated the identification of gene carriers and led to recommendations about screening and prophylactic treatment.

The current study involves data on over 6,000 individuals from 147 families with mismatch repair (MMR) genes. 21.1% of these families had at least one case of pancreatic cancer. These authors reported that the cumulative risk of pancreatic cancer in families with Lynch syndrome was 1.31% up to the age of 50 years. The risk increased to 3.68% by age 70 years.

These authors stated: “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.”

Comments: These data help define the risk of pancreatic cancer in families with Lynch syndrome.

Reference:

[1] Kastrinos F, Mukerhee B, Tayob N, et al. Risk of pancreatic cancer in families with Lynch syndrome. Journal of the American Medical Association. 2009;302:1790-1795.