Hereditary cancer syndromes and pancreatic cancer

This page was reviewed under our medical and editorial policy by

Eric Fowler, MS, LCGC, Manager, Clinical Genetics, Western Region & Licensed Certified Genetic Counselor

This page was reviewed on September 21, 2022.

Genetic conditions that increase cancer risks are called hereditary cancer syndromes. How much these conditions increase the risk of cancer and where in the body the cancer develops are different, depending on which syndrome you have.

Is pancreatic cancer hereditary?

Most pancreatic cancers are not inherited. In 2022, an estimated 62,210 adults (32,970 men and 29,240 women) in the United States will be diagnosed with pancreatic cancer, according to the American Cancer Society. About 10 percent, or one of every 10 of these people, will have hereditary or familial pancreatic cancer.

Familial pancreatic cancer (FPC) refers to families with a high rate of pancreatic cancer. Specifically, those with at least two first-degree relatives with pancreatic cancer or three members of the family who have pancreatic cancer are thought to have a genetic or familial link to the disease.

These genetic syndromes are caused by hereditary errors, called mutations, in the genetic code or DNA. Sometimes these mutations lead to cells growing uncontrollably and becoming cancerous tumors.

The pancreas is an organ in the abdomen that plays a vital role in metabolism. It releases hormones that help the body regulate how much sugar is in the blood and tells the body when to turn energy into fat and store it, or when to burn calories for fuel from stored fat. It also creates and releases digestive juices, which helps the body digest carbohydrates and fats.

About 60,430 new cases of pancreatic cancer will be diagnosed in 2021, and about 48,220 pancreatic cancer patients will die in 2021, according to the National Cancer Institute (NCI) Surveillance, Epidemiology, and End Results (SEER) database. About 11 percent of patients with pancreatic cancer are still alive five years after diagnosis, according to the SEER five-year relative survival rate. Remember that survival rates don’t predict how long any one person may live, but look at overall rates in large groups of people.

More than half of pancreatic cancer patients are first diagnosed with cancer when the tumor has already spread to distant parts of the body. If the cancer is found early, before it has spread, the five-year survival rate is much higher. About 42 percent of pancreatic cancer patients whose tumors haven’t spread out of the pancreas when they are first diagnosed are still alive five years later, according to SEER data.

For this reason, it’s important for people with a hereditary increased risk of developing pancreatic cancer to have their doctor regularly look for any developing tumors. Regular imaging of the pancreas may help detect these cancers early.

What causes inherited risks for pancreatic cancer?

Inherited mutations in some genes have been linked to an increased risk for pancreatic cancer. These genes include:

  • BRCA1 and BRCA2
  • PALB2, a gene linked to inherited breast cancer
  • p16/CDKN2A and CDK4, genetic mutations associated with familial atypical multiple mole melanoma (FAMMM) syndrome
  • MLH1, MSH2 and EPCAM, genes linked to Lynch syndrome, an inherited condition that raises the risk for several types of cancer
  • STK11 gene, also known as Peutz-Jeghers syndrome, which is linked with polyps in the digestive tract and several other cancers
  • Mutations in the CASR, CFTR, CPA1, CTRC, PRSS1 and SPINK1 genes have been linked to pancreatitis, an inflammatory condition of the pancreas

These are germline mutations, which means they are found in the genetic material or DNA of every cell in the body of offspring. A variant from the germline can be passed from parent to child. Other types of genetic defects are acquired over the course of a lifetime and are not passed down from parent to child.

Is genetic testing available to gauge the risk for pancreatic cancer?

A genetic mutation is found in, at most, 20 percent of families with familial pancreatic cancer, according to American Society of Clinical Oncology, but those with a family history of other cancers may be more likely to have an inherited risk. However, anyone diagnosed with pancreatic cancer should undergo genetic testing to look for inherited mutations as soon as possible after diagnosis. If such testing reveals inherited mutations, this is powerful information to share with family members.

If pancreatic cancer runs in your family, the suggested approach is to undergo annual imaging scans starting at age 50 or when you’re 10 years younger than your youngest relative was when diagnosed. These tests may spot pancreatic cancer early, when more treatment options are typically available.

Participation in genetic or family pancreatic cancer registries is also important. This will help researchers identify genetic mutations linked to familial pancreatic cancer and potentially develop a screening test in the future.

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