People with a family history of pancreas cancer now have a way to accurately predict their chance of carrying a gene for hereditary pancreas cancer and their lifetime risk of developing the disease. Developed by Johns Hopkins Kimmel Cancer Center researchers, the novel computer software tool is designed to help genetic counselors and physicians decide who would most benefit from early screening.
An estimated 10 percent of aggressive and highly fatal cases of the disease are caused by inherited genes. Even if there is a 100 percent chance that an individual carries a pancreas cancer gene, their risk for developing the disease is only 20 to 25 percent over their lifetime, says Alison Klein, Ph.D., assistant professor and director of the National Familial Pancreas Tumor Registry at Johns Hopkins. So, while its a rare disease, the need for screening in these persons is important.
The risk calculator, based on similar tools for breast and colon cancer, calculates a percentage score of probability that a person carries a pancreas cancer gene. Called PancPRO, it also computes an individuals lifetime risk of developing the disease.
Although researchers have not yet identified specific genes that cause the disease, they can estimate high risk based on clusters of family members with a history of pancreas cancer. We know how genes behave, and coupled with information about a family - who has the disease, their age, family size, and causes of death - our model can provide a good estimate of an individuals risk, says Klein.
Early risk assessment has long been sought for pancreas cancer that runs in families, Klein says because of the difficulty doctors have diagnosing it before it has already spread. Survival rates are extremely low.
To test the models effectiveness for predicting cancer, Klein and her colleagues fed the software family history information given by more than 6,000 individuals in 961 families when they
Contact: Vanessa Wasta
Johns Hopkins Medical Institutions