Compared to other cancers, pancreatic cancer is relatively rare. But it is the third leading cause of cancer death in the United States.
Recent research and clinical practice insights outline strategies for caring for patients with a family history of pancreatic cancer.
"There is generally an understanding that pancreatic cancer has no effective prevention strategies or screening options," says Sean Mulvihill, MD, pancreatic cancer surgeon at Huntsman Cancer Institute (HCI) and professor of surgery at the Ï㽶ÊÓƵ of Utah (U of U). "Yet recent insights indicate the utility of pancreatic cancer screening for certain patients, and, further, that pancreatic cancer in a patient's family history may be a significant predictor of genetic susceptibility to other cancers, including breast, ovarian, and prostate—all of which have strong screening and prevention options."
Up to 10% of patients with pancreatic cancer have been found to have a hereditary cancer predisposition syndrome. As such, the National Comprehensive Cancer Network recommends genetic testing for all patients with pancreatic cancer or for their close relatives if the individual with pancreas cancer is deceased. Patients found to have a hereditary cancer predisposition usually have an increased risk for multiple cancers:
- Patients with mutations in the BRCA 1/2 genes have an increased risk for breast, ovarian, pancreas, and prostate cancers as well as melanoma.
- Patients with ATM or PALB2 mutations have an increased risk for both breast and pancreas cancers.
- Patients with CDKN2A mutations have an increased risk for both melanoma and pancreas cancers.
HCI's Family Cancer Assessment Clinic offers genetic counseling to pancreas cancer patients and their family members. HCI genetic counselor Cathryn Koptiuch specializes in genetic counseling for people with a family history of pancreas cancer. "This unique service is designed to help patients and health care providers understand cancer risk associated with a family history of pancreatic cancer," says Koptiuch.
HCI genetic counselors help patients with pancreas cancer determine whether a hereditary cancer predisposition could have been responsible for their diagnosis. If so, the patient's relatives can then pursue genetic testing to clarify whether they've inherited the same predisposition to develop cancer (known as cascade testing). This cascade testing approach determines who has increased cancer risks and who does not. Based on their findings, genetic counselors help patients and their families understand how to manage any increased risk or communicate genetic findings to others who may benefit from genetic testing.
In addition to the screening and risk-reducing options that are commonly known for melanoma and breast, ovarian, and prostate cancers, genetic counselors suggest the best pancreas cancer screening plans for patients and their families. These are based on the 2014 CAPS Consortium recommendations as well as genetic literature published since these recommendations were made.
These types of patients may be appropriate for pancreatic cancer screening:
- Patients with a pathogenic mutation in the BRCA1, BRCA2, ATM, or PALB2 gene and who have a first degree relative (sibling, child, or parent) with pancreas cancer
- Patients who have a pathogenic mutation in the CDKN2A or STK11 gene, regardless of their family history
- Patients with a strong family history of pancreatic cancer regardless of their genetic status if their relative’s diagnosis is not known to be hereditary, including the following:
- Patients with two first degree relatives diagnosed with pancreatic cancer
- Patients with two close relatives diagnosed with pancreatic cancer, with one being a first-degree relative to the patient, and both affected relatives being first-degree relatives to each other
Patients undergoing pancreatic cancer screening typically start at age 50 or 10 years prior to the age their relative was diagnosed with pancreatic cancer, whichever comes first. Screening is approached using both annual imaging (MRI and EUS) as well as blood work.
HCI's genetic counselors see patients and their families at HCI in Salt Lake City and throughout the Mountain West via telemedicine at these locations:
- Idaho: Saint Alphonsus Regional Medical Center in Boise and Portneuf Medical Center in Pocatello
- Nevada: Carson-Tahoe Cancer Center in Carson City
- Wyoming: St. John's Medical Center in Jackson and Memorial Hospital of Sweetwater County in Rock Springs
Contact HCI's Family Cancer Assessment Clinic at 801-587-9555 to learn more about genetic testing for patients with a family history of pancreas cancer.