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Hereditary Pancreatic Cancer Information for Patients

In recent years, our knowledge about hereditary pancreatic cancer and genetic testing has increased. While most pancreatic cancer is not hereditary, an estimated 10 percent of pancreatic cancer is due to a hereditary cause.

Hereditary pancreatic cancer is divided into several categories:

  • Known hereditary cancer syndromes caused by a gene(s) that increases the risk for pancreatic and other cancers
  • Pancreatitis, a disease that causes the pancreas to become inflamed, can increase the risk to develop pancreatic cancer. In some cases, chronic pancreatitis is caused by an inherited gene mutation.
  • Clustering of two or more relatives with pancreatic cancer in a family, but the specific cause of cancer risk in these families is currently unknown

Finding a hereditary explanation for why some people developed pancreatic cancer helps to:

  • Provide a reason why they or their relatives were diagnosed with cancer
  • Guide the course of cancer treatment or decision about surgery
  • Clarify the risks for other cancers

Genetic testing is one way to understand if a person has a hereditary risk for pancreatic cancer. If a person has genetic testing and finds that he or she has hereditary pancreatic cancer, it means he or she was born with an increased risk to develop pancreatic and possibly other cancers. Genetic testing for hereditary pancreatic cancer can look for several hereditary cancer syndromes.

In addition, the result of your genetic testing is important information to share with relatives because they may have also inherited the same increased risk to develop cancer. When a hereditary explanation is found in a family, relatives can then better understand their risk to develop cancer, which can help guide their decisions about cancer screening, prevention, and management.

Screening for pancreatic cancer is often performed as part of a research study because we do not yet understand the best way to screen for pancreatic cancer. However, there is some early evidence that pancreatic cancer screening can be of value and considered for people who have a high risk to develop pancreatic cancer. Our Smilow Cancer Hospital experts can discuss the potential benefits, risks, and limitations of pancreatic cancer screening, as well as research studies for people at increased risk for pancreatic cancer.

Hereditary Cancer Syndromes Associated Gene(s) Lifetime Risk of Pancreatic Cancer Other Cancers at Increased Risks
Hereditary Breast and Ovarian Cancer Syndrome (HBOC) BRCA1
BRCA2
Up to 10%, risk varies by gene Breast cancer
Ovarian cancer
Prostate cancer
Melanoma
Familial Atypical Multiple Mole Melanoma Syndrome (FAMMM) CDKN2A Up to 20% Melanoma
Familial Adenomatous Polyposis Syndrome (FAP) APC 2-4% Multiple precancerous colon polyps (totaling 20 or more) Colorectal cancer Gastrointestinal cancers
Lynch Syndrome (Hereditary NonPolyposis Colorectal Cancer or HNPCC) EPCAM
MLH1
MSH2
MSH6
PMS2
Up to 10%, risk varies by gene Colorectal cancer
Uterine and ovarian cancer
Gastrointestinal cancers
Urinary tract cancer
Sebaceous cancer
Peutz-Jeghers Syndrome (PJS) STK11 Up to 36% Gastrointestinal tract polyps
Gastrointestinal cancers
Colorectal cancer
Breast cancer
Gynecological cancers
Other Pancreatic Cancer Syndromes ATM PALB2 5-10% Female breast cancer
Ovarian cancer
Possible prostate cancer
Hereditary Pancreatitis and Pancreatic Cancer Syndromes Associated Gene(s) Lifetime Risk of Pancreatic Cancer Other Increased Risks
Hereditary Pancreatitis (HP) PRSS1
SPINK1
25-40% Multiple events of severe pancreatitis
Cystic Fibrosis (CF) CFTR Not as well defined Chronic lung and pancreatic disease