Familial pancreatic cancer

Familial pancreatic cancer (FPC) is defined as having two or more first degree relatives with pancreatic cancer (PC) not associated with another recognised syndrome.

Pancreatic cancer, specifically pancreatic ductal adenocarcinoma (PDAC), can occur as part of a number of syndromes:

  • Hereditary breast ovarian cancer syndrome
  • Familial atypical multiple mole melanoma syndrome
  • Peutz Jeghers syndrome
  • Lynch Syndrome
  • Li Fraumeni syndrome
  • Hereditary pancreatitis
  • Cystic fibrosis

Risks

An individual’s risk of developing pancreatic cancer increases with the number of affected first degree relatives from 4-7% with only one or two relatives to 17-32% with three or more first degree relatives affected. This risk is increased if a family member has developed PC under the age of 50 years.

Genetics

In the absence of other cancers in the family to suggest the presence of one of the known syndromes associated with PC, genetic testing is not generally offered to FPC families. This may change with the emergence of genetic tests that use panels of cancer susceptibility genes .

Clinical management

PDAC generally has a poor prognosis but the use of endoscopic ultrasound and magnetic resonance imaging have improved the opportunity to detect PC at an earlier stage, with some evidence of an improvement in prognosis. Screening of selected individuals at high risk of developing PC may now be considered appropriate, although evidence is limited. However, there are associated psychological and economic cost implications that need careful consideration (5). Screening for PC should be performed in centres with expertise in detecting and managing the precursor lesions and early stage cancer.

Content updated 18 Oct 2016