Hereditary pancreatic cancers

Family cancer syndromes are rare, and only between 5 and 10 out of 100 pancreatic cancers (5-10%) may be caused by one of these conditions.

Most family cancer syndromes are linked to pancreatic ductal adenocarcinoma, the most common type of pancreatic cancer. A very small number of family cancer syndromes are linked to neuroendocrine tumours (PancNETs) – fewer than 5 in 100 (5%) pancreatic cancers are PancNETs.

Familial pancreatic cancer

Familial pancreatic cancer is pancreatic cancer that runs in families. This includes:

• families with two or more first-degree relatives (parent, brother, sister or child) with pancreatic cancer
• families with three or more relatives with pancreatic cancer on the same side of the family
• families with a known family cancer syndrome and at least one family member with pancreatic cancer.

The risk of familial pancreatic cancer increases if:

• relatives were diagnosed under the age of 60
• there are more than two people under 60 with pancreatic cancer in the family
• there are people with pancreatic cancer in more than one generation on the same side of the family.

We still don’t know what genetic faults cause most familial pancreatic cancers. We only know about the genetic faults that cause about one in five (20%) familial pancreatic cancers.

If you think pancreatic cancer runs in your family, speak to your doctor. If you do have a high risk of familial pancreatic cancer, you may be able to have monitoring.

Hereditary pancreatitis

Pancreatitis is inflammation of the pancreas. Hereditary pancreatitis is a rare condition that runs in families. It starts in early childhood, and can keep coming back. It is linked to a fault in the PRSS1 gene. People who carry this faulty gene have a very high chance of developing pancreatitis.

Pancreatitis can cause severe abdominal (tummy) pain, which often needs treating in hospital. Over time, it may damage the pancreas, which can cause problems, for example with digesting food.

People with hereditary pancreatitis may be more likely to get pancreatic cancer. About two fifths (40%) of people with hereditary pancreatitis may develop pancreatic cancer at some point in their lives. The risk may be higher for people who smoke and people who have diabetes.

It isn’t common for pancreatitis to run in families. Chronic (long-term) pancreatitis that is caused by other things that aren’t inherited (including heavy drinking of alcohol over a long period of time) may also increase the risk of pancreatic cancer.

If you have hereditary pancreatitis, speak to your doctor. You may be able to have monitoring for pancreatic cancer.

Updated August 2018

Review date August 2021